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Fraturas em Crianças
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Pathological Fractures in Children

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Fractures in Children with Preexisting Bone Conditions

When we talk about “pathological fractures in children”, it is essential to clarify that the fracture itself is not pathological, but rather the bone can present a series of changes, such as structural, metabolic, dysplastic or infectious. Therefore, it is more accurate to refer to the bone as pathological, not the fracture itself. Within this context, our focus will be on fractures that occur in children with pre-existing bone conditions.

This chapter aims to address more specifically the fractures that develop in children with such conditions. It is important to highlight that the scope of this topic is vast, which leads us to define the topics to be discussed.

We decided not to include fractures related to infectious processes or metabolic disorders, such as rickets or osteopsatirosis, in this chapter. Instead, our focus will be on stress fractures, considering the differential diagnosis, as well as those arising from pre-existing tumoral or pseudo-tumorous bone lesions.

Stress fractures are particularly relevant due to their nature and challenges associated with diagnosis and treatment. Furthermore, fractures resulting from tumor or pseudo-tumor bone lesions require a specialized approach to ensure appropriate management and the best possible prognosis.

Therefore, in outlining this chapter, we seek to provide a comprehensive view of fractures in children with pre-existing bone conditions, highlighting the most relevant aspects for their understanding and clinical management.

  • Benign Bone Tumors:

Among the benign tumor lesions of childhood, which can most frequently cause fractures, we highlight osteoblastoma and chondroblastoma.

Osteoblastoma – 

Osteoblastoma is a locally aggressive bone tumor that in long bones has a metaphyseal location, initially cortical and eccentric. This injury, as it is locally aggressive, with great destruction of the bone framework, causes micro fractures, due to erosion of the bone cortex (figs. 1 and 2). The progressive destruction of the cortex predisposes to complete fracture, when the involvement exceeds fifty percent of the bone circumference. The fracture of this lesion facilitates local dissemination, making oncological treatment difficult, which requires elaborate reconstructions and there is a limitation in functional recovery (Figs. 3 and 4). 

Figura 1 – aspecto clínico de osteoblastoma do primeiro metacarpeano. Fig. 2 – lesão insuflativa, com erosão da cortical e microfraturas.
Figure 1 – clinical appearance of first metacarpal osteoblastoma. Fig. 2 – Insufflation injury, with cortical erosion and microfractures.
Figura 3 – agressividade local com grande destruição da estrutura óssea, sendo necessária a reconstrução com enxerto autólogo do ilíaco, apesar da baixa idade. Fig. 4 – aspecto clínico e funcional, pós-operatório.
Figure 3 – local aggressiveness with great destruction of the bone structure, requiring reconstruction with an autologous iliac graft, despite young age. Fig. 4 – clinical and functional appearance, post-operatively.
Osteoblastoma of the spinal pedicle can cause analgesic scoliosis, due to the pain of the tumor process or the fracture (fig. 5 and 6).
Figura 5 – escoliose antálgica devido à osteoblastoma. Fig. 6 – fratura do pedículo pelo tumor ósseo.
Figure 5 – Antalgic scoliosis due to osteoblastoma. Fig. 6 – fracture of the pedicle caused by the bone tumor.

Chondroblastoma –

Chondroblastoma affects the epiphyseal region of growing long bones (figs. 7 and 8) and, less frequently, the apophyseal portion (figs. 9 and 10).

Figura 1 – condroblastoma epifisário do fêmur.
Figure 1 – Epiphyseal chondroblastoma of the femur.
Figura 2– erosão da cartilagem articular.
Figure 2– erosion of the articular cartilage.
3 – condroblastoma da apófise do grande trocânter. Fig. 4 – aumento significativo da lesão, com fratura arrancamento do grande trocânter.
3 – chondroblastoma of the apophysis of the greater trochanter. Fig. 4 – significant increase in the lesion, with a tearing fracture of the greater trochanter.

This bone tumor causes resorption of the epiphysis (or apophysis), erosion of the bone cortex and joint invasion, leading to arthralgia, which can cause deformity and joint subsidence fracture.

The treatment of both osteoblastoma and chondroblastoma is surgical and must be carried out as soon as possible, as these lesions, despite being histologically benign, quickly progress to destruction of the local bone framework.

The best indication to avoid local recurrence is segmental resection. However, due to the articular location of the chondroblastoma, it is preferable to provide adequate surgical access to each region, as in this example that affects the posteromedial region of the femoral head (fig. 11), to perform careful intra-lesional curettage, followed by local adjuvant , such as phenol, liquid nitrogen or electrothermia (fig. 12), to subsequently fill the cavity with an autologous bone graft, restoring the anatomy of the region (fig. 13) and reestablishing function (figs 14 and 15). 

Figura 7 – via de acesso à região póstero-medial da cabeça femoral.
Figure 7 – access route to the posteromedial region of the femoral head.
Figura 8 – curetagem intralesional, seguida de eletrotermia.
Figure 8 – Intralesional curettage, followed by electrothermia.
Figura 9 – enxerto ósseo autólogo já integrado. Fig. 10 – Função de flexão com carga dos quadris.
Figure 9 – autologous bone graft already integrated. Fig. 10 – Load-bearing flexion function of the hips.
Figura 11 Abdução do quadril, após um ano da cirurgia.
Figure 11 Hip abduction, one year after surgery.
Chondroblastoma, despite being a benign lesion, in addition to local recurrence, can evolve into pulmonary metastases (fig. 16 and 17) which remain histologically benign, making the indication of additional chemotherapy controversial.
Figura 12 – radiografia do tórax, com múltiplos nódulos.
Figure 12 – chest x-ray, with multiple nodules.
Figura 13 – tomografia do tórax, com treze anos de evolução.
Figure 13 – Chest tomography, with thirteen years of evolution.

 In our experience we had two cases of osteoblastoma and one case of chondroblastoma with secondary lung disease. In this case of chondroblastoma, thoracotomy was performed and numerous pulmonary nodules were found, which persist to this day. This patient, at the time of diagnosis of metastases, presented with hypertrophic pulmonary osteopathy. He did not undergo any complementary treatment and is asymptomatic to this day, thirteen years later (fig. 18 and ’19) and fifteen years after surgery (fig. 20 and 21).

Figura 14 – Abdução apos 8 anos. Fig. 15 – Fexão com carga após 13 anos.
Figure 14 – Abduction after 8 years. Fig. 15 – Loaded bending after 13 years.
Figura 16 – Abdução após 15 anos. Fig. 17 Flexão com carga após quinze anos da cirurgia.
Figure 16 – Abduction after 15 years. Fig. 17 Flexion with weight fifteen years after surgery.

  • Malignant Bone Tumors:

The most common malignant bone neoplasms in childhood are osteosarcoma and Ewing’s sarcoma, which must be diagnosed at the onset of symptoms, as they cause pain and a palpable tumor and need to be treated early.

Osteosarcoma –

In our country, it is not uncommon for osteosarcoma to present with a fracture at diagnosis (figs. 22 and 23).  

Figura 22 – osteossarcoma da metáfise distal do fêmur. Fig. 23 – fratura e disseminação local ao diagnóstico.
Figure 22 – Osteosarcoma of the distal metaphysis of the femur. Fig. 23 – fracture and local spread at diagnosis.
In these situations, local oncological control may require ablative surgery, with Van-Ness gyroplasty (figs. 24 and 25) being an alternative to be considered.
Figura 24 – incisão rombóide para a cirurgia de Van-Ness.
Figure 24 – rhomboid incision for Van-Ness surgery.
Figura 25 – ressecção ampla, com margem oncológica, preservando-se o feixe vasculo-nervoso.
Figure 25 – wide resection, with oncological margin, preserving the vascular-nervous bundle.
This surgery is an interim amputation that changes the function of the ankle. This undergoes a 180 degree rotation and will act as if it were the knee, with the aim of transforming an amputation at thigh level into a “below the knee” amputation. Functionally, it acts as if it were an amputation of the leg, with the terminal support of the calcaneus and without the need for a mechanical “knee” (figs. 26 and 27). Special orthoses need to be made to fit the patient (fig. 28 and 29). There is a need for social and psychological support for the success of this procedure, which is little accepted and therefore rarely recommended in our country.
Figura 26 – giroplastia de Van-Ness, apoio terminal no calcâneo. Fig. 27 – a contratura dos ísquio tibiais, suturados nos flexores dorsais do tornozelo, realizam a flexão.
Figure 26 – Van-Ness gyroplasty, terminal support on the calcaneus. Fig. 27 – the contracture of the tibial ischium, sutured to the ankle's dorsal flexors, performs flexion.
Figura 28 – a contratura do quadríceps, suturado no tendão calcâneo, realiza a extensão do “neo joelho”. Fig. 29 – órteses especiais para adaptação. Há necessidade de fisioterapia especializada e apoio psicológico e social ao paciente e aos familiares.
Figure 28 – quadriceps contracture, sutured to the Achilles tendon, extends the “neo knee”. Fig. 29 – special orthoses for adaptation. There is a need for specialized physiotherapy and psychological and social support for the patient and family.

Ewing sarcoma – 

Ewing’s Sarcoma is a malignant bone tumor that can be confused with osteomyelitis and can be diagnosed after a fracture (figs 30 to 36).  

Figura 30 – Sarcoma de Ewing após fratura e quimioterapia. Fig. 31 – Ressecção do tumor ósseo e reconstrução biológica com enxerto ósseo autólogo.
Figure 30 – Ewing sarcoma after fracture and chemotherapy. Fig. 31 – Resection of the bone tumor and biological reconstruction with autologous bone graft.
Figura 32 – Reconstrução com fíbula e enxerto autólogo de crista ilíaca.
Figure 32 – Reconstruction with fibula and autologous iliac crest graft.
Figura 33 – Pós operatório de dois meses, em quimioterapia adjuvante. Fig. 34 – Após três anos.
Figure 33 – Two months post-operative, undergoing adjuvant chemotherapy. Fig. 34 – After three years.
Figura 35 – Após onze anos da cirurgia. Fig. 36 – Após 22 anos do tratamento.
Figure 35 – Eleven years after surgery. Fig. 36 – After 22 years of treatment.

Click here to see the full case  

In children, cases of malignant neoplasms that lead to fractures are fortunately rare.

Pseudotumorous Bone Lesions:

The bone lesions that most frequently accompany fractures in children are pseudo-tumor lesions, with emphasis on simple bone cysts, aneurysmal bone cysts, fibrous dysplasia and eosinophilic granulomas, in this order of frequency.

Eosinophilic granuloma –

Eosinophilic granuloma presents as a local inflammatory condition and a lesion with bone rarefaction accompanied by a thick lamellar periosteal reaction, which is a radiographic characteristic of benignity. Another aspect of eosinophilic granuloma is that it presents an area of ​​bone rarefaction without corresponding extra-osseous involvement (fig. 37), distinguishing it from Ewing’s sarcoma, which is the tumor that presents the earliest extra-cortical tumor.

Eosinophilic granuloma can present with a clinical picture of fracture when it affects the spinal column where a wedging fracture of the vertebral body occurs, described as Calvé’s vertebra plano (fig. 38). In this situation, this injury may progress to spontaneous healing, and restoration of the vertebra body may even occur.

Other locations where micro-fractures can occur are when they affect the supra-acetabular region (fig. 39), or in load-bearing areas such as the proximal metaphyseal portion of the femur (fig. 40), due to cortical erosion. medial.  

37 – reação periosteal lamelar grossa em granuloma eosinófilo. Não há lesão extra-óssea. Fig. 38 – vértebra plana de Calvé (fratura acunhamento do corpo vertebral em granuloma eosinófilo).
37 – thick lamellar periosteal reaction in eosinophilic granuloma. There is no extra-osseous injury. Fig. 38 – Calvé plano vertebra (wedge fracture of the vertebral body in eosinophilic granuloma).
Figura 39 – granuloma eosinófilo do ilíaco – fratura afundamento do acetábulo. Fig. 40 – Fratura incompleta da cortical medial do fêmur, devido à granuloma eosinófilo.
Figure 39 – eosinophilic granuloma of the iliac – sinking fracture of the acetabulum. Fig. 40 – Incomplete fracture of the medial cortex of the femur, due to eosinophilic granuloma.

This lesion responds well to simple curettage surgical treatment, with the need to add a bone graft being exceptional.

Click here to read more.

Fibrous Dysplasia –

Fibrous dysplasia is a pseudo-tumor lesion that most frequently leads to bone deformity. However, when it affects the femur, it can cause a previous deformity, like a shepherd’s crook, characteristic of this condition, with consequent fracture (fig. 41). The femoral neck region with fibrous dysplasia often develops a fracture, even without previous deformity (fig. 42). 

To correct the defect, it is necessary to curettage the lesion, fill it with autologous bone graft and corrective osteotomies of the deformity (fig. 43). A fracture in this location can be difficult to resolve, due to the difficulty in consolidation due to the dysplastic appearance of the bone (fig. 44), leading to recurrence of the disease and deformity.

Figura 41 – Deformidade em cajado de pastor, seguida de fratura. Fig. 42 – fratura do colo femoral, em displasia fibrosa, sem deformidade prévia.
Figure 41 – Deformity in a shepherd’s crook, followed by fracture. Fig. 42 – fracture of the femoral neck, in fibrous dysplasia, without previous deformity.
Figura 43 – osteotomia corretiva, com enxerto autólogo. Fig. 44 – doença poli cística do colo femoral, por displasia fibrosa.
Figure 43 – corrective osteotomy, with autologous graft. Fig. 44 – polycystic disease of the femoral neck, due to fibrous dysplasia.
Fibrous dysplasia can be polyostotic (fig. 45) and be part of the MacCune-Albhright syndrome, characterized by fibrous dysplasia, precocious puberty and skin patches (fig. 46). Fractures can occur even without significant trauma, due to bone fragility (fig. 47).
Figura 45 – lesão no fêmur, por displasia fibrosa.
Figure 45 – injury to the femur, due to fibrous dysplasia.
Figura 46 – Mancha café com leite, em síndrome de MacCune-Albhright.
Figure 46 – Café-au-lait spot, in MacCune-Albhright syndrome.
Figura 47 – Fratura da ulna, devido à displasia fibrosa.
Figure 47 – Fracture of the ulna, due to fibrous dysplasia.
This disease tends to stabilize after puberty (fig. 48 and 49), but sometimes several surgical procedures are necessary during growth (fig. 50 and 51) to avoid compensatory deformities and achieve successful treatment. treatment.
48 – recidiva da deformidade, em síndrome da Albright .
48 – recurrence of the deformity, in Albright syndrome.
Figura 49 – criança em desenvolvimento com recidiva .
Figure 49 – developing child with relapse.
Figura 50 – nova recidiva da deformidade, necessidade de reoperação .
Figure 50 – new recurrence of the deformity, need for reoperation.
Figura 51 – estabilização da doença displásica após a adolescência .
Figure 51 – stabilization of dysplastic disease after adolescence.

Fibrous dysplasia may also be part of congenital pseudoarthrosis, which most frequently affects the distal third of the tibia, but can occur in other locations such as the proximal third of the tibia (figures 52, 53 and 54), with all the difficulties in reaching it. if consolidation.

Figura 52 – Deformidade proximal da tíbia em displasia fibrosa. Fig. 53 – Fratura
Figure 52 – Proximal tibial deformity in fibrous dysplasia. Fig. 53 – Fracture
Figura 54 – Aspecto cirúrgico do foco displásico de pseudo-artrose, em displasia fibrosa.
Figure 54 – Surgical appearance of the dysplastic focus of pseudoarthrosis, in fibrous dysplasia.

Congenital pseudoarthrosis is a condition that deserves to be studied in a separate chapter.

Aneurysmal Bone Cyst –

The aneurysmal bone cyst, also called multilocular hematic cyst, is a lesion of insufflative bone rarefaction filled with serosanguineous fluid, interspersed with spaces varying in size and separated by septa of connective tissue containing trabeculae of bone or osteoid tissue and ostoclastic giant cells ( figs. 55 and 56).

Figura Figura 55 – Cisto ósseo aneurismático da tíbia. As lojas ocorrem em número e tamanho variados, aglomerando-se e provocando erosão do trabeculado ósseo, que se expandem e insuflam a cortical.
Figure Figure 55 – Aneurysmal bone cyst of the tibia. The stores occur in varying numbers and sizes, clumping together and causing erosion of the bone trabeculae, which expand and inflate the cortex.
Figura Figura 56 – A tomografia revela área radiolucente; erosão óssea; afilamento da cortical e insuflação. sem focos de calcificação.
Figure Figure 56 – Tomography reveals a radiolucent area; bone erosion; cortical thinning and inflation. no foci of calcification.

The patient generally presents with mild pain at the site of the injury, when the affected bone is superficial, and inflammatory signs such as increased volume and heat may be observed. Generally, the patient correlates the onset of symptoms with some trauma.  

In evolution there may be a slow, progressive or rapidly expansive increase. It affects any bone, most frequently the lower limbs, tibia (figs. 57 and 58) and femur representing 35% of cases.

Figura Figura 57 – COA metafisário da tíbia com insuflação da cortical, erodindo a placa de crescimento.
Figure 57 – Metaphyseal AOC of the tibia with cortical inflation, eroding the growth plate.
Figura Figura 58 – aspecto homogênio com erosão da cortical
Figure 58 – homogeneous appearance with cortical erosion

The vertebrae are also affected by this injury, including the sacrum. In the pelvis, the iliopubic branch is most frequently affected. They can mimic joint symptoms when they reach the epiphysis. Compromise in the spine can cause compressive neurological symptoms, although in most cases it affects the posterior structures.

         The treatment of choice has been marginal resection or intra-lesional curettage, followed by filling the cavity with an autologous or homologous graft, when necessary. The cavity can also be filled with methylmethacrylate, although our preference is to use an autologous graft when possible, as it is a benign lesion. Some authors associate intralesional adjuvant treatment with the application of phenol, electrothermia or cryotherapy. In classic aneurysmal bone cysts, I do not see the point of this therapy, which, however, should be applied when the surgeon finds a “suspicious” area that was not detected on imaging. If the aforementioned benign tumors are involved, which may be accompanied by areas of aneurysmal bone cyst, local adjuvant therapy will be beneficial.

Some bone segments such as the ends of the fibula, clavicle, rib, distal third of the ulna, proximal radius, etc. can be resected, without the need for reconstruction.

In other situations, we may need segmental reconstructions with free or even vascularized bone grafts or joint reconstructions with prostheses in advanced cases with major joint involvement. In the spine, after resection of the lesion, arthrodesis may be necessary to avoid instability.

Radiotherapy should be avoided due to the risk of malignancy, however it may be indicated for the evolutionary control of lesions in difficult to access locations, such as the cervical spine, for example, or other situations in which surgical re-intervention is not recommended.

Embolization as an isolated therapy is controversial. However, it can be used preoperatively to minimize bleeding during surgery. This practice is most used in cases of difficult access, although its effectiveness is not always achieved. Infiltration with calcitonin has been reported with satisfactory results in isolated cases.

Recurrence may occur, as the phenomenon that caused the cyst is unknown and we cannot guarantee that surgery repaired it. The recurrence rate can reach thirty percent of cases.

Simple Bone Cyst –

A simple bone cyst is a pseudo-tumor lesion that can occur in any part of the skeleton and most frequently presents with fracture (figures 59 to 64).

Figura 59 – fratura metafisária do úmero em C.O.S..
Figure 59 – metaphyseal fracture of the humerus in COS.
Figura 60 – microfratura do rádio em C.O.S..
Figure 60 – microfracture of the radius in COS.
Figura 61 – fratura infracção do acetábulo em C.O.S..
Figure 61 – Infraction fracture of the acetabulum in COS.
Figura 62 – Fratura completa do colo femoral em C.O.S..
Figure 62 – Complete fracture of the femoral neck in COS.
Figura 63 – Fratura do fêmur em C.O.S..
Figure 63 – Femur fracture in COS.
Figura 64 – Fratura da tíbia, após entorse em C.O.S..
Figure 64 – Tibial fracture, after COS sprain.

A simple bone cyst can occasionally be diagnosed due to an increase in volume, but when it presents a painful symptom, it is generally related to micro fractures or often a complete fracture.

            The humerus is the most affected bone. Micro-fractures can eventually provide partial “cure” in some areas of the cyst and with growth the metaphysis moves away from the lesion, which begins to occupy the diaphyseal zone (fig. 65 and 66). This progression to the diaphysis can occur asymptomatically and a new painful clinical manifestation may occur acutely (fig. 67).

Figura 65 – Cisto ósseo metafisário do úmero.
Figure 65 – Metaphyseal bone cyst of the humerus.
Figura 66 – cicatrização da região metafisária, crescimento ósseo e deslocamento diafisário da lesão remanescente.
Figure 66 – healing of the metaphyseal region, bone growth and diaphyseal displacement of the remaining lesion.
Figura 67 – aumento da lesão e fratura.
Figure 67 – increase in injury and fracture.
Bone cysts in older children and distant from the growth plate are considered mature cysts, which can heal with different treatment methods, including after the occurrence of a fracture (fig. 68 to 71).
Figura 68 – COS da fíbula. Fig. 69 – Fratura em COS.
Figure 68 – COS of the fibula. Fig. 69 – Fracture in COS.
Figura 70 crescimento ósseo metafisário e “migração” da lesão em direção à diáfise. Fig. 71 Cicatriz de COS que foi distanciando-se da placa de crescimento que teve cura expontânea.
Figure 70 metaphyseal bone growth and “migration” of the lesion towards the diaphysis. Fig. 71 COS scar that distanced itself from the growth plate that healed spontaneously.

In these situations, the treatment adopted must be appropriate for the bone and the fracture in question, and may be closed or open, with the indication of filling with a bone graft depending only on the specific needs of the fracture, when surgical treatment is indicated. 

In mature bone cysts, the complete fracture causes great decompression of the lesion and consolidation and healing of the lesion can be achieved simultaneously. However, in some cases, there is a need for additional treatment of the cyst, after consolidation of the fracture, when closed treatment is chosen (fig. 72 to 78).

Figura 72 – Fratura diafisária do úmero, em cisto ósseo maduro (cisto distante da placa de crescimento em adolescente). Fig. 73 – Consolidação após imobilização com tipóia.
Figure 72 – Diaphyseal fracture of the humerus, in a mature bone cyst (cyst distant from the growth plate in an adolescent). Fig. 73 – Consolidation after immobilization with a sling.
Figura 74 – Refratura. Fig. 75 – Novo tratamento incruento.
Figure 74 – Refracture. Fig. 75 – New bloodless treatment.
Figura 76 – Consolidação e persistência de áreas císticas.
Figure 76 – Consolidation and persistence of cystic areas.
Figura 77 Calo de fratura e áreas císticas.
Figure 77 Fracture callus and cystic areas.
Figura 78 – Função após múltiplas fraturas.
Figure 78 – Function after multiple fractures.
In our infiltration technique, we usually evaluate the cavity by injecting contrast, aiming to verify whether the cyst is unicameral or whether it has septa forming gaps that would require an individual approach. We began to observe through radioscopy the presence of contrasted vascular flow when contrast was injected into the cyst. We believe that there is an intraosseous pseudo-aneurysm that, when swirling, causes cystic erosion (fig. 79 to 86).
Figura 79 – Diagnóstico do cisto após fratura proximal do fêmur. Fig. 80 – Tratamento incruento, com tração cutânea por seis semanas
Figure 79 – Diagnosis of the cyst after a proximal femur fracture. Fig. 80 – Bloodless treatment, with skin traction for six weeks
Figura 81 – Consolidação da fratura, com cura parcial da lesão cística. Fig. 82 – Tratamento do cisto remanescente com infiltração. Observa-se contraste evidenciando a presença vascular na fisiopatologia desta lesão.
Figure 81 – Consolidation of the fracture, with partial healing of the cystic lesion. Fig. 82 – Treatment of the remaining cyst with infiltration. Contrast is observed, highlighting the vascular presence in the pathophysiology of this lesion.
Figura 83 – Cisto ósseo consolidado, após fratura e tratamento com infiltração. Presença de lesões císticas remanescentes.
Figure 83 – Consolidated bone cyst, after fracture and infiltration treatment. Presence of remaining cystic lesions.
Figura 84 – COS da patela, corte sagital.
Figure 84 – COS of the patella, sagittal section.
Figura 85– cisto unicameral da patela, corte axial.
Figure 85– unicameral cyst of the patella, axial section.
Figura 86 – contraste demonstra a alteração vascular, com verdadeiras fístulas arterio- venosas na fisiopatologia.
Figure 86 – contrast demonstrates the vascular alteration, with true arteriovenous fistulas in the pathophysiology.
Some locations, such as the femoral neck, deserve special attention and should preferably be operated on before a fracture occurs (fig. 87 and 88).
Figura 87 – Cisto ósseo simples no colo femoral, antes da fratura. Fase de tratamento mais simples
Figure 87 – Simple bone cyst in the femoral neck, before fracture. Simplest treatment phase
Figura 88 – Fratura do colo femoral em cisto ósseo
Figure 88 – Fracture of the femoral neck in a bone cyst
Treating this injury after a fracture requires specific and complex planning to be successful (fig. 89 to 94).
Figura 89 – Preparo do orifício da placa angulada.
Figure 89 – Preparation of the angled plate hole.
Figura 90 – Orifício adaptado para permitir orientação do parafuso paralelo à lâmina.
Figure 90 – Hole adapted to allow screw orientation parallel to the blade.
Figura 91 – Preparação do enxerto.
Figure 91 – Graft preparation.
Figura 92 – redução da fratura, posicionamento do enxerto e fixação.
Figure 92 – fracture reduction, graft positioning and fixation.
Figura 93 – pós-op um ano.
Figure 93 – one year post-op.
Figura 94 – rx perfil. Fig. 95 – função após 1 ano.
Figure 94 – profile x-ray. Fig. 95 – function after 1 year.

Click here to see the full case

Stress Fracture –

 Stress fractures deserve special attention in this article both because they are more frequent than reported in the literature, as many cases go unnoticed, and because of the florid appearance that imaging studies portray, causing difficulty in differential diagnosis.

The child complains of pain, usually after physical exertion, which, as it is mild, ends up resolving spontaneously.

However, an orthopedist may be consulted and, when requesting an x-ray, be surprised by a periosteal reaction in the metaphyseal region in a growing patient.

The concern about the possibility of osteomyelitis, eosinophilic granuloma, osteosarcoma or Ewing’s sarcoma is justified, but it is necessary to be aware of clinical aspects, such as time of evolution, improvement factors, local appearance, so as not to complicate this diagnosis, which is  clinical. radiological  (fig. 96 and 97).

Figura 96 – dor há um mês .
Figure 96 – pain for a month.
Figura 97 – reação periosteal.
Figure 97 – periosteal reaction.
Carrying out other tests such as bone mapping (fig. 98) and computed tomography (fig. 99) confirm the existence of the lesion, but may not be sufficient to clarify the diagnosis.
Figura 98 –hipercaptação.
Figure 98 – hypercapture.
Figura 99 – TC com produção de osso
Figure 99 – CT with bone production

It is necessary to evaluate and ask:  in the time it took to carry out these tests, was there no clinical improvement?

Magnetic resonance imaging is an exam that needs to be interpreted very carefully, as the fracture causes intra- and extra-osseous edema that can scare less experienced people.

We must observe the detail of the two low signal points of the fracture callus in the lateral and medial cortex in figure 100 as well as the low signal point of the bone callus in the posterior cortex in figure 101.

Figura 100 – RM em fratura de stress.
Figure 100 – MRI in stress fracture.
Figura 101 – Baixo sinal do calo ósseo
Figure 101 – Low signal from bone callus

The inflammatory process of the fracture, with marked hemorrhage and edema, is extensive. The histology of the fracture callus may mimic osteosarcoma. There is a known case of amputation due to an erroneous diagnosis of osteosarcoma in a patient with a stress fracture.

            Observation for two to three weeks is essential for an accurate diagnosis and is not considered bad practice, even in neoplasms. The x-ray taken three weeks later showed the stress fracture (fig. 102 and 103) and the clinical picture with improvement in symptoms and reduction in edema reaffirms the diagnosis. The clinic is sovereign.

Figura 102 – Rx após 3 semanas.
Figure 102 – Rx after 3 weeks.
Figura 103 – A linha da fratura é nítida.
Figure 103 – The fracture line is clear.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

Phone: +55 11 3231-4638  Cell:+55  11 99863-5577   Email:  drpprb@gmail.com

Fratura em osso patológico
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Pathological bone fracture

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Pathological bone fracture

Pathological bone fracture. In our experience dedicated to the study and treatment of patients with bone diseases, we have experienced numerous cases of fractures that hid undiagnosed diseases.

Pathological bone fracture. The simplified designation of “pathological fracture” is not appropriate, as every fracture is a pathological process. It is correct to use the term: pathological bone fracture,  which is most often related to neoplasms, whether primitive or metastatic.

The pathological processes that can lead to fractures are classified as bone dysplasias, circulatory disorders, degenerative, inflammatory and infectious or neoplastic changes.

For the correct diagnosis, it is necessary to consider the patient’s clinical aspects, fracture mechanisms, imaging, laboratory and anatomopathological aspects.

Bone fractures, which hide undiagnosed pathological processes, can result in inadequate orthopedic management.

We will organize them didactically within the five chapters of General Pathology, namely: dysgenesis or dysplasias, degenerative processes, circulatory disorders, inflammations and neoplastic diseases.

  • BONE DYSPLASIAS:

–  dis  (from the Greek = alteration),  plasien  (= form). Any change in bone morphology, whether congenital or hereditary, can cause deformities and/or fractures. Due to the frequency and polymorphism of the anatomical changes they present, we highlight the following:

1 –  Osteopsatirosis or Osteogenesis imperfecta:

In any of its manifestations, in Rubin’s classification, it is a hereditary disease that predominates in the diaphysis of long bones and determines changes in bone morphology due to deficient sub-periosteal bone apposition. Longitudinal bone growth occurs at the level of the epiphyseal line, where cartilage transforms into bone tissue. Transverse growth, however, depends on endosteal resorption and subperiosteal bone apposition. Failure of this modeling mechanism in bones leads to impaired growth in the transverse direction. Due to this pathogenesis, the bones become very thin and fragile, subject to frequent fractures. 

Figura 1 – Radiografias com múltiplas fraturas, calos exuberantes e deformidades, freqüentes nesta doença
Figure 1 – Radiographs showing multiple fractures, exuberant calluses and deformities, common in this disease
Figura 2 – peça anatômica de fêmur de paciente autopsiado, portador desta afecção
Figure 2 – anatomical piece of femur from an autopsied patient, suffering from this condition
Figura 3 – Desmineralização acentuada e deformidade, alargamento do canal medular.
Figure 3 – Marked demineralization and deformity, enlargement of the spinal canal.

2 –  Osteopetrosis or Albers Schomberg Disease:

It is a disease characterized by changes in the epiphyseal line of bones of endochondral origin. The lesions are condensing due to the failure in the activity of osteoclasts which, under normal conditions, act in the physiological resorption of bones. With apposition prevailing over resorption, the bones initially condense in the metaphysis and, progressively, throughout the entire bone, whose consistency becomes stony. In addition to anemia, which results from the reduction and even absence of marrow spaces, the seat of hematopoiesis, denser bones lose their elasticity and can fracture.

3 –  Fibrous dysplasia, mono or polyostotic:

It is a condition in which there is partial replacement of the bone by fibrous proliferation between osteoid beams with little mineralization and has lower radiographic density. With growth and skeletal maturation, progressive ossification generally occurs, which may even resemble normal bone structure. The injured area has a lower density than that of normal bone and, therefore, the main anatomical manifestation is deformity, which sometimes leads to fracture.

  • METABOLIC CHANGES  :

For bones to maintain a normal structure, the apposition and reabsorption mechanisms must be in balance. Apposition depends on the activity of osteoblasts that elaborate the 

collagen fibers, protein matrix of bones. Along the collagen fibers there will be deposition of minerals, tricalcium phosphate, in the form of hydroxyapatite crystals. Protein collagen fibers account for 95% of the structure of the bone matrix. The remaining 5% are mucopolysaccharides, hyaluronic and chondroitinsulfuric acid, which predominate in the “cement lines” or “reverse lines”, which delimit the different bands of matrix apposition, maintaining normal bone growth. Simultaneously, bone resorption is carried out by osteoclasts under stimulation of parathyroid hormone. These mechanisms of apposition and reabsorption, which represent the so-called bone remodeling ( turnover ), are intense in the first decade of life, less in the second and progressively less with advancing age, but always present throughout our lives.

Normal bone metabolism, therefore, consists of: a- apposition of the protein matrix whose collagen fibers are produced by osteoblasts, which require muscular activity to fulfill their functions; b- dietary protein intake, vitamins A and C, minerals mainly calcium and phosphorus; c- gonad, thyroid, pituitary and adrenal hormones are also necessary for matrix formation and mineralization. For reabsorption, osteoclasts produce enzymes that enable the dissolution of the matrix and the solubilization of minerals, which act under the stimulus of parathyroid hormone.

Changes in any of the elements that contribute to altering turnover will lead to  metabolic bone disease,  especially the following:

1 –  Osteoporosis:

It is an important and frequent cause of fractures, caused by reduced bone consistency due to the quantitative reduction of the matrix, reducing the mineral deposit bed that leads to greater bone fragility and fractures, especially of the vertebrae and femur. Osteoporosis does not depend on a lack of calcium or phosphates, as it means matrix deficiency, which reduces the area of ​​mineral apposition. The causes arise from less muscular activity in people with a sedentary lifestyle, particularly the elderly or in patients who have been bedridden for a long time, hence the increasing importance of exercise to treat it, in addition to a diet with adequate protein intake. States of protein deficiency due to dietary deficiency or excess elimination are subject to osteoporosis, as occurs in states of malnutrition and diseases with dysproteinemia, such as multiple myeloma and bone carcinomatosis. Osteoporosis also occurs in changes in endocrine glands, such as postmenopausal hypoestrogenism, hyperthyroidism, pituitary adenomas, gigantism and acromegaly, changes in the adrenal cortex, which lead to Cushing’s syndrome, and others.

2 –  Osteomalacia and rickets:

These are diseases resulting from mineral deficiency, that is, they do not depend on changes in the protein matrix. In rickets, mineral deficiency predominates in the epiphyseal lines or growth plates, where the demand is greatest and necessary for the mineralization of the newly formed osteoid beams. Due to the lower resistance of these regions, there will be a “cup-shaped” enlargement in the metaphyses of the long bones and a “rickety rosary” in the ribs. Osteomalacia is also known as “adult rickets”. Although uncommon, it translates into generalized mineral deficiency, as there is no growth plate in the bones. Occurs due to reduced intestinal absorption 

in patients who have undergone major intestinal resections or due to dietary deficiency. Fractures result from greater bone fragility caused by mineral deficiency.

3 –  Hyperparathyroidism:

 An important cause of pathological bone fracture, often the initial sign of this disease, especially in its primary form, the cause of which is the adenoma of one of the parathyroid glands. Parathormone normally acts on osteoclasts, cells that carry out bone reabsorption. It also acts on the kidneys, inhibiting the tubular reabsorption of phosphates and, in this way, exerts control over phosphaturia and, consequently, over phosphaemia. When there is an excess of parathyroid hormone, there will be hyperphosphateria, altering the Ca/P balance which, under normal conditions, maintains a 2:1 ratio, from the blood (9.5 calcium / 4.5 mg phosphorus) to the hydroxyapatite formula. There will, therefore, be hypercalcemia to maintain blood Ca/P balance. Calcium is removed from the bones, which are the largest depository of this mineral in our body, normally retaining around 95% of this mineral. As a consequence of this process, the bones will become more fragile with spontaneous fractures or due to mild trauma. Another important sign of the disease is recurrent calculosis, especially kidney stones. Primary hyperparathyroidism is a long-term chronic disease that, if not treated with extirpation of the parathyroid adenoma, will fatally lead to progressive and generalized demineralization of the bones with multiple fractures and intraosseous cystic formations, due to the intensity of reabsorption by osteoclasts. Furthermore, the presence of so-called “brown tumors”, isolated or multiple, is common, the pathogenesis of which is due to intraosseous hemorrhages with the presence of ferric hemosiderin pigment, in addition to clusters of osteoclasts. All of these bone changes result in demineralization of the beams and partial replacement by fibrosis, progressing to the so-called  generalized fibrocystic osteitis  or Von Recklinghausen’s disease of the bones, which should not be confused with neurofibromatosis, which also bears the name of this author.

Figura 4 Tumor marron do hiperparatireoidismo
Figure 4 Brown tumor of hyperparathyroidism
Figura 5 Lesão na falange, fratura e tumor da paratireóide.
Figure 5 Injury to the phalanx, fracture and parathyroid tumor.
Figura 6 Regeneração óssea após osteossíntese e paratireoidectomia.
Figure 6 Bone regeneration after osteosynthesis and parathyroidectomy.

  • DEGENERATIVE DISEASES:

Pathological bone fracture

Within this chapter we can include  Langerhans cell histiocytoses  , called Histiocytoses X, by Lichtenstein, and lipidoses.

1 – Langerhans cell histiocytosis:

-including eosinophilic granuloma and Hand Schiller-Christian disease.

  • Eosinophilic granuloma:

The most frequent form is  Eosinophilic Granuloma  , which is more common in children, is generally monostotic and is characterized by an osteolytic lesion in the cranial vault in the form of a circular “bite-shaped” lesion, in the vertebral body and in the diaphysis of long bones. When located in the vertebra, it compromises the body of this bone, with osteolysis and “collapse”, flattening the vertebral body, which constitutes a fracture with radiographic appearance of the so-called flat vertebra of Calvè. In long bones, it affects the diaphysis and, depending on the size of the lesion, fractures may occur.

Figura 7 lesão osteolítica na calota craniana, freqüente localização e achatamento do corpo vertebral (vértebra plana de Calvé.
Figure 7 Osteolytic lesion in the cranial vault, frequent location and flattening of the vertebral body (Calvé's flat vertebra.
Figura 8 Vértebra plana de Calvè – Lesão no úmero
Figure 8 Flat vertebra of Calvè – Injury to the humerus

  • Hand – Schuller – Christian disease:

In  Hand – Schuller – Christian disease , which may be the evolution of eosinophilic granuloma, the lesions are multiple with severe bone involvement, due to the clusters of macrophages that are frequently xanthomylated, due to the accumulation of cholesterol esters. These accumulations can also compromise the pituitary bed and the retro-ocular region, which can lead to the symptomatic triad, that is, multiple osteolytic lesions, diabetes insipidus and unilateral or bilateral exophthalmos. Long bones are often the site of fractures.

2 –  Gaucher disease:

Among lipidoses, it is the form that most compromises bones. In this entity, histiocytic cells, due to an enzymatic defect, are full of lipids that replace the structure of the bones, especially in the femurs, constituting an important cause of necrosis of the head of this bone, accompanied by deformities, which can lead to fractures.

  • CIRCULATORY DISORDERS   :

In bone pathology, the most significant example of intraosseous blood circulation disorder occurs in  Paget’s disease,  also known as  osteitis deformans,  described in 1892(), by Sir Thomas Paget, and until today considered to be of unknown cause.

1 – Paget’s disease:

Paget’s disease occurs in advanced age groups, generally over 50 years of age, mono or polyostotic. In the initial stages of this disease, there is a considerable increase in intraosseous arterial circulation which, as it is active, arterial, there is marked bone reabsorption with radiological and anatomopathological lesions that present characteristics of the so-called “circumscribed osteoporosis”, more frequent in the skull, pelvis, femur and tibia. At this stage, fractures may occur due to the greater fragility of the bones. The most frequent symptoms are pain and discomfort in the affected area. Some authors report cases in which intraosseous circulation is up to 100 times greater than normal, which can progress to heart failure. It is a slowly evolving disease with deformities and, due to the progressive increase in density, the bones assume a stony consistency. The anatomopathological substrate shows disorder in the bone apposition and resorption mechanisms, demonstrated histologically by the numerical increase in the cement lines that demarcate the increasingly greater apposition bands in the compromised bone. These lines become so evident that they assume a “mosaic arrangement”, with a progressive, disordered numerical increase in osteoclasts and osteoblasts on the margins of the bone beams, which become irregular, interspersed with fibrosis in the inter-trabecular spaces. “Chalk fractures” result from greater bone density and less elasticity, which is why they have a straight line, similar to broken chalk.

Figura 9 Fratura em giz ( traço transverso)
Figure 9 Chalk fracture (transverse line)
Figura 10 linhas de cimento formando mosaico
Figure 10 cement lines forming mosaic

2 – Blood Dyscrasias:

In  blood dyscrasias  such as leukemia or hemolytic diseases such as anemia (sickle cell, spherocytic and Cooley). They are rare, but circulatory disorders can occur, with extensive bone infarctions, causes of pathological bone fractures.

  • INFLAMMATIONS :

Inflammations in general are divided into two large groups:  non-specific , in which the arrangement of the cells does not allow the etiological agent to be identified, and  specific , or granulomatous, in which the cellular arrangement allows the etiology to be identified, as in tuberculosis.

1 – Hematogenous Osteomyelitis:

Hematogenous osteomyelitis stands      out among the nonspecific processes, more common in children and adolescents. These mainly affect long bones, most frequently in the metaphyses of the femur and tibia. The location is due to blood stasis in the epiphyseal lines where demand is intense, making it suitable for the development of bacteria. Due to the intensity of the inflammatory process, although infrequent, fractures may occur.

2 – Tuberculosis:

Tuberculosis    is a  specific process, whose osteolytic lesion can lead to fractures. When located in the spine, Pott’s disease, the disease compromises the intervertebral spaces, with secondary osteolysis that can result in fractures with wedging of the vertebrae with subsequent kyphosis.

3 – Deep Mycoses:

Among the deep mycoses,  South American blastomycosis , whose agent is  paracoccidioidis brasiliensis , although uncommon in bone location, is the one that most likely causes fractures.

4 – Parasites:

Echinococus  Granulosus,  among the parasites, is the one that causes most bone fractures .  It is a rare disease in our country, known as hydatid cyst. In our experience we had two cases, one of them with a severe osteolytic vertebral lesion and the other, a femoral lesion, which also fractured. Treatment is surgical.

  • NEOPLASMS:

Regardless of whether they are benign or malignant, tumors can cause fractures, depending on the aggressiveness of the bone structure. Bones with greater overload, such as the vertebrae and those of the lower limb, are more prone to fractures than the others. Benign tumors include  osteoblastoma, enchondroma, chondromyxoid fibroma, gigantocellular tumor and hemangioma.

1 – Osteoblastoma:

Osteoblastoma  is a  neoplasm that is more aggressive, which is why it manifests with osteolysis and can be the cause of fractures, most frequently in long bones or the spine.

2 – Enchondroma:

Enchondroma  , which in approximately 50% of cases is present in the phalanges of the hands, although benign and often asymptomatic, can manifest itself as a spontaneous or traumatic fracture. When located in long bones, mainly in the humerus and femur, they can also cause fractures and must be differentiated from bone infarction using imaging methods, sometimes only clarified with a biopsy of the lesion.

3 – Giant Cell Tumor – TGC:

The  gigantocellular tumor is most common in the epiphysis of long bones, mainly distal to the femur and proximal to the tibia and humerus. The possibility of fracture arises from the frequency with which it extends to the metaphysis, sometimes with high local aggressiveness.

4 – Chondromyxoid Fibroma:

Chondromyxoid fibroma ,  most common in the femur and tibia, is slow-growing, eccentric in relation to the bone axis, and can rarely be the cause of fracture.        

5 – Hemangioma:

Hemangioma  , in long bones or the spine, can be asymptomatic and is sometimes diagnosed by chance finding in a radiographic examination carried out for other reasons. Under certain conditions, however, it manifests itself as a fracture and/or “collapse” of the vertebrae body. It can be isolated or multiple, characterizing bone hemangiomatosis.

6 – Osteosarcoma – Chondrosarcoma:

All  primitive malignant bone neoplasms  present the possibility of fracture. The ones that most commonly do this are the osteolytic forms of  osteosarcoma , mainly teleangectatic.

7 – Malignant hemangioendothelioma:

malignant hemangioendothelioma  , due to the  intensity of vascularization and resulting intraosseous hemorrhages.

8 – Plasmacytoma / Multiple myeloma:

Osteolytic lesions of  plasmacytoma/myeloma , caused by intramedullary clusters of atypical plasma cells, are often the first sign of the disease.

9 – Lymphoma:

Other lytic tumors such as   intraosseous lymphomas .

10 – Fibrosarcoma / Malignant Fibrohistiocytoma:

Less common is  fibrosarcoma ,  malignant fibrous histiocytoma.

11 – Liposarcoma:

Liposarcoma  , the most common soft tissue tumor, can also present with a fracture .

  • Bone Metastases – Breast, Prostate, Lung, Kidney and Thyroid Cancer: 

The main manifestation of pathological bone fractures is due to  secondary neoplasms or metastases  (from the Greek: meta=beyond, stasis=stop). The most frequent bone metastases in men originate from the prostate and lungs. In women, they are of breast and pulmonary origin. Those originating from prostate carcinoma are generally osteocondensant, because, due to the slowness with which the cells reach the vertebrae, through the para-vertebral venous plexus of Batson and in the other bones via arterial blood, the bone tissue reacts with neoformation of inter-trabecular beams that reduce the medullary spaces, in order to condense the bone, clearly evident in imaging methods or pathological examination. As in Paget’s disease, the greater density and less elasticity of bones can cause “chalk line fractures”. Lung metastases, in both sexes, are osteolytic with more frequent involvement of the humerus, pelvis and femur. In the spine, the lesion initially affects the pedicles, while in plasmacytoma/myeloma the involvement predominates in the vertebral body. Breast carcinoma metastases are generally osteolytic. Osteolytic fractures have a pathogenesis based on the greater speed with which cells reach the bone, preventing an adequate osteogenic reaction, contrary to what is observed in prostatic carcinoma. Other neoplasms, originating in the kidney (clear cell carcinoma) and thyroid (follicular carcinoma), due to the intense vascularization that is part of these structures, quickly destroy bone tissue resulting in intensely osteolytic bone fractures, sometimes clinically pulsatile.

Figura 11 Destruição óssea pela neoplasia
Figure 11 Bone destruction due to neoplasia

  • Pseudotumor Lesions – Simple Bone Cyst, Aneurysmal Cyst and Non-Ossifying Fibroma:

As for  pseudo-neoplastic lesions , the one that most frequently causes fractures is the  aneurysmal bone cyst . This process, of unknown etiology, which does not have a cystic appearance and much less is vascular in nature, is known as the benign lesion that has the most aggressive behavior, often simulating malignant neoplasms.

Other pseudo-neoplastic lesions that can fracture are the  simple bone cyst  of metaphyseal location, when in a bone with greater load such as the femur and tibia, it is prone to fracture. Non-ossifying fibroma ,  an evolution of the cortical metaphyseal fibrous defect, can also fracture due to its progressive increase in volume, when located in the metaphysis of the femur or tibia.

It is not uncommon for simple or complex bone fractures to hide pathological changes and may result in inadequate orthopedic treatment.

To treat pathological bone fractures, it is necessary to study the entire context that surrounds them. Bone fractures must always be analyzed under multidisciplinary aspects, which take into account the history, age group of the patients, clinical aspects, images, laboratory tests and anatomopathological examination. The joint multidisciplinary study of these data is essential for the diagnosis and management of each case. With the correct diagnosis, the orthopedist will define the treatment. Following what is described in this chapter:

Bone Dysplasias:

1 –  Osteopsatirosis or Osteogenesis imperfecta.

Treatment : Clinical: The use of bisphosphonates is currently used.

                    Orthopedic: intramedullary osteosyntheses to support aligned growth, with telescoped rods, associated or not with osteotomies to correct deformities.

2 –  Osteopetrosis

Treatment : Clinical: Prevention of deformities

                    Orthopedic: osteosynthesis of fractures

3 –  Fibrous dysplasia, mono or polyostotic,

Treatment : Clinical: The use of bisphosphonates may have an effect.

                    Orthopedic: osteosynthesis

Metabolic Changes:

1 –  Osteoporosis

Treatment : Clinical: prevention of fractures, avoid caffeine, walking. The use of bisphosphonates may be indicated.

                    Orthopedic: osteosynthesis of fractures

2 –  Osteomalacia and rickets

Treatment : Clinical: Correction of homeostasis, vitamin D, prevention of deformities.

                    Orthopedic: osteosynthesis of fractures.

3 –  Hyperparathyroidism

Treatment : Clinical: resection of the parathyroid tumor and compensation of the metabolic condition, taking into account the marked hypocalcemia that occurs after surgery, as the bone tissue begins to quickly compensate for the existing bone demineralization. Protein supply is essential for the creation of the bone matrix.

                    Orthopedic: osteosynthesis of fractures, which heal quickly, as the bone is starved for calcium.

Degenerative Diseases:

1 – Eosinophilic granuloma

Treatment : Clinical: corticosteroid therapy

                    Orthopedic: curettage of the bone lesion. In Calvé’s flat vertebra, the wedging fracture itself leads to healing of the process. In children and adolescents, the vertebral body spontaneously grows, correcting the deformity.

2 –  Gaucher disease

Treatment : Clinical:

                    Orthopedic: 

Circulatory disorders:

1 – Paget’s disease

Treatment : Clinical: Bisphosphonates and anti-inflammatories.

                    Orthopedic: osteosynthesis of fractures

2 – In  blood dyscrasias

Treatment : Clinical:

                    Orthopedic:

Inflammations:

1 –    Hematogenous osteomyelitis

Treatment : Clinical: antibiotic therapy

                    Orthopedic: drainage of abscesses, removal of bone sequestra and stabilization of fractures.

2 –    Tuberculosis

Treatment : Clinical: triple regimen for tuberculosis.

                    Orthopedic: cleaning of caseous abscesses and immobilization, with arthrodesis of the affected joints and osteosynthesis of fractures often being indicated.

3 –  South American Blastomycosis , whose agent is  paracoccidioidis brasiliensis  and

Treatment : Clinical: specific drug treatment for ringworm

                    Orthopedic: surgical cleaning and specific care for each case.

4 –  Echinococcosis,  in the form of a hydatid cyst, must be treated surgically.

Neoplasms:

1 –  Benign primitives : Orthopedic treatment can include intralesional curettage, local adjuvant, reconstruction with osteosynthesis and autologous graft or methyl methacrylate.

2 –  Malignant Primitives : May require neoplasia-oriented chemotherapy treatment and surgical treatment of resection and reconstruction with endoprostheses or biological reconstruction, if possible, or ablative surgery.

3 –  Secondary to metastases : Restoring function is essential for the patient’s quality of life. The treatment option for these fractures requires some consideration to make the appropriate choice for each patient. Some of them are subjective, as we have to assume the patient’s likely survival time, clinical possibility that they will fully recover their functions, ability to withstand anesthesia, etc.

            We seek to support our decisions on the following parameters:

  1. Is the injury to the upper limb, lower limb, pelvic girdle or spine?
  2. Is the injury single or are there multiple injuries?
  3. Has the fracture already occurred or is there a risk of fracture?
  4. If no fracture occurred, is 1/3 of the bone’s circumference already compromised? Does the injury cover a long area?
  5. Did the patient walk before the fracture, did he have normal motor function?
  6. How long has the patient been treated for the primary disease?
  7. Are you currently receiving chemotherapy treatment?
  8. What is this patient’s temporal prognosis?
  9. What co-morbidities do you have in addition to the neoplasm?
  10. The type of primary neoplasm responds to radiotherapy

The analysis of these issues will allow a therapeutic decision to be made that assists the patient in recovering their motor function, in line with the treatment of their underlying disease.

Patients with myeloma have a high rate of postoperative infections and usually benefit from local radiotherapy, especially in lesions of the thoracic spine or upper limb, in the first years of the disease. After a few years, when the disease becomes refractory to chemotherapy or bone marrow transplantation, even surgical options are limited due to the intensity of bone involvement.

This case in figures 8 to 12 exemplifies a patient with multiple myeloma, presenting an extensive lesion in the proximal half of the right humerus. Despite being myeloma, which responds well to chemotherapy and radiotherapy and even in the upper limb, there is an indication for resection of the lesion and reconstruction with a non-conventional endoprosthesis due to the destruction of the anatomy and providing prompt restoration of function.

Figura 12: Destruição completa do 1/3 proximal do úmero. Figura 13: Reconstrução com endoprótese.
Figure 12: Complete destruction of the proximal 1/3 of the humerus. Figure 13: Reconstruction with endoprosthesis.
Figura 14: Aspecto estético. Figura 15: Recuperação funcional no pós operatório imediato. Figura 16: Função com carga.
Figure 14: Aesthetic appearance. Figure 15: Functional recovery in the immediate post-operative period. Figure 16: Loaded function.

Injuries to the lower limb, as it is a load-bearing limb, are best resolved with surgical treatment. The use of palliative radiotherapy, considering a “reserved prognosis”, can cause more suffering when the lesion fractures, as all neoplasms cause the replacement of normal bone tissue by tumor tissue. Therefore, there is bone lysis in all neoplasms, including prostate metastases. It is often mistakenly said that prostate bone metastases are osteoblastic, but what happens pathophysiologically is that in slow-evolving neoplasms there is time for the bone tissue to react to the injury, in an attempt to repair the bone that has been injured, or lysed. if you prefer.

In figures 13 to 19, we illustrate a case of breast cancer metastasis in a patient who had a mastectomy two months ago. We observed numerous lytic lesions in the proximal metaphyseal region of the left femur on December 23, 1987. Local radiotherapy was recommended. The injury did not respond to treatment and in this location, lower limb, in just 40 days the injury progressed and fractured, increasing the patient’s pain and the family’s discomfort. 

The lesion was resected and replaced with an endoprosthesis.

Figura 17: Lesões líticas em novembro de 1987. Figura 18: Destruição e fratura proximal do fêmur.
Figure 17: Lytic lesions in November 1987. Figure 18: Destruction and proximal fracture of the femur.
Figura 19: Reconstrução com endoprótese.
Figure 19: Reconstruction with endoprosthesis.

After one year and six months, a lesion appears in the ilio-pubic branch and in the femoral neck on the right side and we should not wait for it to fracture but rather treat it prophylactically.

Figura 20: Após 18 meses lesão no colo femoral direito.
Figure 20: After 18 months injury to the right femoral neck.
Figura 21: Operada antes de fraturar. Reconstrução bilateral com endoprótese.
Figure 21: Operated before fracturing. Bilateral reconstruction with endoprosthesis.
Figuras 22 e 23: Carga monopodal nos dois membros inferiores. Qualidade de vida para o paciente e cuidadores.
Figures 22 and 23: Single-leg load on the two lower limbs. Quality of life for the patient and caregivers.
Methods for treating fractures offer numerous osteosynthesis options, but in their techniques the tumor lesion is not resected. If the patient’s survival is short, this palliative option can resolve the issue, even with limitations, but if the patient is active, with a short period of neoplastic disease, the local lesion that has not been resected can evolve and cause pain and functional disability for the patient. , which will require re-operation in worse conditions and with greater technical difficulties.
In this last example, a single lesion is observed in the femoral shaft. He was treated with a blocked femoral nail. We observed the progression of the injury each month, complaining of pain and difficulty walking that progressively worsened. Note the local destruction and instability of osteosynthesis, figures 20 to 26.
Figura 24: Cintilografia detectando apenas uma lesão. Figura 25: Pequena lesão diafisária de metástase de câncer renal.
Figure 24: Scintigraphy detecting only one lesion. Figure 25: Small diaphyseal lesion from kidney cancer metastasis.
Figura 26: Osteossíntese, sem ressecção da lesão. Figura 27: Progressão da lesão, dor e incapacidade de caminhar.
Figure 26: Osteosynthesis, without resection of the lesion. Figure 27: Progression of the injury, pain and inability to walk.
iguras 28 e 29: Aumento acentuado do tamanho da lesão, dor e impotência funcional.
Figures 28 and 29: Marked increase in the size of the lesion, pain and functional impotence.
Figura 30: Necessidade de reoperação. Cicatriz da nefrectomia-seta vermelha e cicatriz do bloquei da haste-seta amarela.
Figure 30: Need for reoperation. Nephrectomy scar-red arrow and rod block scar-yellow arrow.

The patient underwent re-operation, with the locking screws and femoral stem removed, the injured segment resected and reconstructed with a diaphyseal prosthesis.

Figura 31: Retirada do parafuso de bloqueio.
Figure 31: Removing the locking screw.
Figura 32: Mensuração do tamanho da endoprótese diafisária.
Figure 32: Measurement of the size of the diaphyseal endoprosthesis.
Figura 33: Cimentação do componente proximal.
Figure 33: Cementation of the proximal component.
Figura 34: Cimentação do componente distal.
Figure 34: Cementation of the distal component.
Figura 35: União e cimentação dos dois componentes.
Figure 35: Union and cementing of the two components.
Figura 36: Radiografia de controle. Figura 37: Função restabelecida. Carga autorizada no pós operatório imediato.
Figure 36: Control x-ray. Figure 37: Function reestablished. Load authorized in the immediate post-operative period.

The patient can walk from the first day after surgery, being able to return to their work activity and complementary treatment of the underlying disease.

These examples illustrate the difficulties in approaching pathological fractures and the need for professionals with experience in treating these injuries.

See fracture of the femoral neck in a child due to a simple bone cyst.

See incomplete fracture of the femoral neck in an adult due to renal metastasis.

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PROSPERO, JD. in Bone Tumors. Ed. Roca, São Paulo, Brazil, 2001, pgs.211-226

RUBIN, P: Dynamic classification of bone dysplasias, Year Book Medical publisher Inc. Osteogenesis imperfecta pg.322 -324

RUBIN, P.: Dynamic classification of bone dysplasia, Year Book Medical publishers Inc., 1964. Osteopetrosis pg. 258 – 280 

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

Phone: +55 11 3231-4638  Cell:+55  11 99863-5577   Email:  drpprb@gmail.com

Fibroma Condromixóide: Neoplasia Condromixóide Óssea
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Chondromyxoid Fibroma

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Chondromyxoid Fibroma: Chondromyxoid Neoplasm of Bone

Chondromyxoid Fibroma: Bone Chondromyxoid Neoplasia is a rare lesion in bone tissue, which manifests itself in the metaphysis of long bones in an eccentric manner. Characterized by a lobulated appearance, it presents an internal halo of bone sclerosis that separates it from the surrounding normal tissue, often accompanied by cortical erosion, denoting a certain local aggressiveness. The presence of calcifications within it is a common feature of all cartilaginous lesions.

From a histological point of view, Chondromyxoid Fibroma exhibits notable cellular pleomorphism, with the presence of areas of chondroid, fibrous tissue and a significant amount of myxoid material, often accompanied by multinucleated giant cells.

This type of injury may also be associated with an aneurysmal bone cyst, being most commonly found in the proximal metaphysis of the tibia, mainly affecting adolescents and young adults.

The standard treatment for Chondromyxoid Fibroma is surgery. Generally, the approach involves partial parietal resection of the lesion, accompanied by local adjuvant measures, such as the use of phenol, electrothermia or liquid nitrogen, in addition to bone grafting when necessary. In more advanced cases, segmental resection may be indicated. Curettage can also be used, especially in joint regions, but it must be carried out carefully to avoid recurrences.

In short, Chondromyxoid Fibroma is an uncommon bone lesion, but it requires an appropriate surgical approach to avoid complications and guarantee a satisfactory recovery for the patient.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

Phone: +55 11 3231-4638  Cell:+55  11 99863-5577   Email:  drpprb@gmail.com

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Diagnosis of Tumors

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Diagnosis of Tumors

Parameters to guide the diagnosis of tumors:

1. Introduction:

If neoplasia is suspected, the patient must be initially analyzed with clinical assessment, laboratory tests, imaging methods and anatomopathological examination. This multidisciplinary study is necessary for an accurate diagnosis, which will allow appropriate management in each case.

Data such as sex, age and location, associated with plain radiography, are the initial parameters, which allow the first diagnostic hypotheses. Computed tomography and magnetic resonance imaging, as well as scintigraphy, should be performed to assess the location, extent, number of lesions and their relationships with neighboring structures.

Diagnosis of tumors

2. Parameters:

We must analyze the following aspects of the injury:

1) Identify the compromised bone or bones;

2) Regarding the number of injuries:  

          2.1) Located in a bone: monotopic;

          2.2) A lesion in several bones: monotopic and polyostotic;

          2.3) Multiple lesions in one bone: polytopic and monostotic;

          2.4) Multiple lesions in different bones: polytopic and polyostotic.

3) Regarding location in the bone:

          3.1) Epiphysis, metaphysis or diaphysis;

          3.2) Cortical, spongy, subperiosteal, paraosteal or juxta-cortical region;

          3.3) Central or eccentric.

4) Limits of bone injury:

          4.1) Precise, imprecise, infiltrative or permeative, surrounded or not by reactional sclerosis;

          4.2) It goes beyond the cortex with an extra-osseous lesion;

          4.3) It reaches the soft tissues (yes/no) (displaces/infiltrates);

          4.4) Exceeds the growth line.

5) Regarding other aspects of the injury:

          5.1) Destructive (osteolytic)

          5.2) Condensing or osteogenic

          5.3) Multiloculated, “in soap bubbles”

          5.4) Calcifications: focal, diffuse, striated

6) Type of periosteal reaction:

          6.1) In thin slices – “in onion skins”

          6.2) In thick sheets

          6.3) Spiculates – “in sunbeams” or “in a comb”

          6.4) Periosteal survey interrupted by the tumor – Codman’s Triangle

 

3. Diagnosis:

 Study methods for pathological anatomical examination:

 Cytology:

It is the study of desquamated cells obtained from secretions, excretions or obtained with needles and making “imprints” (printing tissue fragments on slides). It should rarely be used to diagnose bone neoplasia. Its importance lies mainly in the cytohistological correlation.

Punch-biopsy:

Collection of material with trephines for inclusion in paraffin and microscopic examination. Although the material obtained by this method is small, when it is collected from a significant area of ​​the neoplasia and by an orthopedist with experience in handling these lesions, it makes a definitive diagnosis possible. The location for obtaining this material must be planned by the surgeon, in order to prevent disruption of the tumor’s balance in neighboring tissues, preventing its spread.

Incisional biopsy:

It is the most used method for diagnosing bone tumors. The biopsy site must be planned, not only in terms of the area that will enable a better histological diagnosis but also to predict future resection of the tumor, which should include the skin of the biopsied region. The biopsy should not be performed in inappropriate locations of the tumor, such as areas of necrosis, hemorrhage, Codman’s triangle or in areas that only present peritumoral reactional bone sclerosis.

Frozen biopsy

It is performed during the surgical procedure. This method is not recommended when there is bone tissue. The possibility of a diagnostic error is high in this situation. Diagnostic errors in numerous bone lesions with multinucleated giant cells, in the various tumors of undifferentiated cells, small cells and round cells, the impossibility of a histological differential diagnosis when there is neoformed bone tissue in the fracture callus, osteosarcoma and myositis ossificans, are some examples that contraindicate the method. Frozen examination may be useful in cases of metastatic lesions and even so, the speed of the method will not alter the operative approach.

Microscopic study:

Fragments obtained by puncture or incisional biopsy must be embedded in paraffin and subsequently stained with hematoxylin-eosin. Special methods such as PAS (Periodic Acid Schiff) and silver impregnation to study reticulin are usually used for differential diagnosis, for example, between Ewing Sarcoma, Lymphomas and PNET (Primitive Neuroectodermal Tumor). PAS, demonstrating glycogen and a scarce amount of reticulin, are common for diagnosing Ewing’s sarcoma. In Lymphomas, reticulin is abundant and PAS is negative. Immunohistochemistry techniques with immunoperoxidase are entering the routine of anatomopathological examinations. They are mainly indicated in the search for the diagnosis of the organ of origin of metastatic neoplasms in the bones. The use of markers that allow identifying the origin of the neoplastic cell is increasingly used in daily practice. Examples are PSA, to identify neoplasia originating from the prostate, CK7 for primitive lung neoplasia, CK20 for primitive digestive tract neoplasia and estrogen and progesterone receptors for breast neoplasia.

Surgical parts:

Routinely a surgical specimen must be examined externally and at the cuts. Externally for analysis of surgical margins in order to verify whether the neoplasm was completely extirpated. In the sections, we verified the involvement of the bone, extension and dimensions of the neoplasm and its main macroscopic characters for adequate microscopic study. (Figure 1) 

When the study of a surgical resection is of a patient undergoing preoperative chemotherapy, particularly in osteosarcoma and Ewing’s sarcoma, the study of the specimen must follow a systematized examination, as the purpose is to analyze the response of the neoplasm to therapy. The study stages will be as follows:

A) Slices will be made of the surgical piece along its entire length with a maximum thickness of 0.5 cm,

B) One or more slices must be reproduced on a computer “scanner” or photographed and x-rayed,

C) This reproduction must be gridded from the proximal to the distal end,

D) The fragments from each checkered area must be thoroughly examined under a microscope in order to quantify the necrosis of the neoplasm and the persistence of histologically viable tumor cells,

E) The final report of the study of the entire specimen must be graded according to the response to preoperative chemotherapy according to the Huvos criteria.

Huvos Criteria:

Grade I: Up to 50% tumor necrosis;

Grade II: 50 to 90% tumor necrosis;

Grade III: Above 90% necrosis;

Grade IV: 100% tumor necrosis – Absence of histologically viable neoplastic cells.

With this degree, the oncologist will be able to guide post-operative treatment taking into account the worst statistical prognosis in cases of cranes I and II and better in those of III and IV.

Video: Diagnosis of bone injuries

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

Phone: +55 11 3231-4638  Cell:+55  11 99863-5577   Email:  drpprb@gmail.com

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Chondrosarcoma

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Chondrosarcoma: History, Clinical Aspects. History: In 1920, the Bone Sarcoma Registry Committee of the American College of Surgeons, composed of Ewing, Codman and Bloodgood, published the first systematic classification of bone tumors. This classification encompassed a wide variety of clinicopathological entities that were basically subdivided into:

Chondrosarcoma: History, Clinical Aspects, Classification, Differential Diagnosis, Treatment, Complication and Prognosis

– primary tumors of the bones 
– tumors developed in pre-existing bone lesions 
– tumors resulting from ionizing radiation
– tumors that invade the bones, originating from soft tissues
– primary tumors of the joints
– metastatic tumors in the skeleton 23 .

     In 1925, Keiller and later Phemister, 1930, were the first to separate chondrosarcoma from osteogenic sarcomas, considering the distinction between their morphological and clinical-radiological characteristics, as well as their slower evolution and better prognosis. In 1939 the “Committee of the bone sarcoma registry” included chondrosarcoma as a distinct entity.

   Lichtenstein and Jaffe, in 1943, established a clear distinction between osteosarcoma and chondrosarcoma. Osteosarcoma is a tumor that produces neoplastic osteoid, while chondrosarcoma occurs from fully developed cartilage, often presenting calcification or ossification, but never producing neoplastic osteoid 2 .

    Definition : Chondrosarcoma is a malignant neoplasm of mesenchymal nature, producing interstitial substance and cells that take on the appearance of hyaline cartilage, with varying degrees of immaturity and frequent foci of calcification. It is the most common primary malignant lesion of the bone after osteosarcoma 23,24  and Ewing’s tumor (silabus), it can affect any age, with a predominance between 30 and 40 years 7,11,22 , with reports in the literature between three and 73 years 15 .

    Chondrosarcoma occurs in bones of endochondral origin, mainly in the roots of the limbs: shoulder (Figures 1 to 3), pelvis (Figures 4 to 10), ribs and axial skeleton 1 , being rare in bones of membranous origin 11,14,15 ,24.

    In most cases they are painless and do not cause motor deficits. The occurrence of fractures is uncommon 7,10,13,22  and patients seek treatment many years after the appearance of the tumor, as symptoms appear late. Huvos 11  reported a case of rib chondrosarcoma that progressed for fifteen years before the patient sought treatment.

    Figures 1 to 10 illustrate large chondrosarcomas of the shoulder girdle and pelvic girdle, which evolved slowly. 

Figura 1: Condrossarcoma com lesão na borda medial da escapula.
Figura 2: Extensão do tumor para a região anterior e axilar.
Figure 1: Chondrosarcoma with lesion on the medial border of the scapula. Figure 2: Extension of the tumor to the anterior and axillary region.
Figura 3: No perfil proeminência superior da lesão. Neoplasia de crescimento lento e muitas vezes indolor.
Figure 3: In profile, superior prominence of the lesion. Slow-growing and often painless neoplasm.
Figura 4: Grande condrossarcoma implantado no ramo íliopúbico
Figure 4: Large chondrosarcoma implanted in the iliopubic branch
Figura 5: Corte da peça ressecada, evidenciando as margens e o tecido neoplásico irregular, de aspecto cartilaginoso, com áreas de calcificação e necrose tumoral.
Figura 5: Corte da peça ressecada, evidenciando as margens e o tecido neoplásico irregular, de aspecto cartilaginoso, com áreas de calcificação e necrose tumoral. Figura 6: Radiografia após ressecção, com função normal do quadril.
Figure 5: Section of the resected piece, showing the margins and irregular neoplastic tissue, with a cartilaginous appearance, with areas of calcification and tumor necrosis. Figure 6: Radiograph after resection, with normal hip function.
Figura 7: Outro exemplo no ilíaco - radiografia com lesão na pelve esquerda. Figura
8: Tomografia com focos de calcificação.
Figure 7: Another example in the iliac - x-ray with injury in the left pelvis. Figure 8: Tomography with calcification foci.
Figura 9: Ressonância T1 evidenciando grande volume tumoral intra-pélvico.
Figura 10: Captação de contraste pela lesão.
Figure 9: T1 MRI showing a large intra-pelvic tumor volume. Figure 10: Contrast uptake by the lesion.

    Classification:  They can be classified according to  location, histology and origin.

    As for location, they can be:

A- central  (Figures 11 to 15);
B- juxtacortical, paraosteal, or periosteal 2,6,23,24  (Figures 16 and 17);
C- peripheral or exophytic , which occurs on an osteochondroma  28  (Figures 18 and 19) and
D- soft tissue 13  (Figures 20 to 31).

Figura 11: Tumor central, com focos de calcificação, erosão da cortical e alargamento do canal medular.
Figure 11: Central tumor, with foci of calcification, cortical erosion and enlargement of the medullary canal.
Figura 12: Ressonância magnética com focos de calcificação.
Figura 13: Forte captação de contraste no local da lesão.
Figure 12: Magnetic resonance imaging with foci of calcification. Figure 13: Strong contrast uptake at the lesion site.
Figura 14: Corte da peça cirúrgica evidenciando tumor que infiltrou os tecidos moles, pelo trajeto da agulha de biópsia.
Figure 14: Section of the surgical specimen showing tumor that infiltrated the soft tissues, along the path of the biopsy needle.
Figura 15: As células cartilaginosas nutrem-se por embebição, implantando-se com facilidade. Em detalhe a disseminação iatrogênica do tumor.
Figure 15: Cartilaginous cells are nourished by imbibition, implanting themselves easily. In detail the iatrogenic dissemination of the tumor.
Figura 16: Radiografia de condrossarcoma justacortical, com grande tumor provocando impressão na cortical lateral do úmero.
Figure 16: Radiograph of juxtacortical chondrosarcoma, with a large tumor causing an impression on the lateral cortex of the humerus.
Figura 17: Corte da peça evidenciando lesão cartilaginosa com erosão da cortical. O periósteo contorna externamente o tumor.
Figure 17: Section of the piece showing cartilaginous injury with cortical erosion. The periosteum externally surrounds the tumor.
Figura 18: Condrossarcoma periférico do fêmur esquerdo, secundário à malignização de osteocondromatose. Observe os osteocondromas na região metafisáriado fêmur direito (um pediculado na face lateral e outro séssil medialmente).
Figure 18: Peripheral chondrosarcoma of the left femur, secondary to malignant osteochondromatosis. Note the osteochondromas in the metaphyseal region of the right femur (one pedicled on the lateral side and the other sessile medially).
Figura 19: Na ressonância magnética o condrossarcoma periférico secundário à osteocondromatose do fêmur esquerdo apresenta heterogeinicidade e intensa captação de contraste tanto na periferia como no interior da lesão.
Figure 19: On magnetic resonance imaging, peripheral chondrosarcoma secondary to osteochondromatosis of the left femur shows heterogeneity and intense contrast uptake both at the periphery and inside the lesion.
Figura 20: Condrossarcoma de tecidos moles da mão.
Figura 21: Apresentando baixo sinal em T1.
Figure 20: Soft tissue chondrosarcoma of the hand. Figure 21: Showing low signal on T1.
Figura 22: Alto sinal em DP e imagem heterogênea com realce intenso pelo contaste em T1.
Figura 23: Com supressão de gordura e contraste (gadolíneo).
Figure 22: High signal on PD and heterogeneous image with intense contrast enhancement on T1. Figure 23: With fat suppression and contrast (gadoline).
Figura 24: Condrossarcoma de tecidos moles na face lateral da coxa esquerda, próximo à patela (abaulamento lateral).
Figura 25: O paciente realizou biópsia em outro Hospital, por incisão horizontal, inadequada.
Figure 24: Soft tissue chondrosarcoma on the lateral aspect of the left thigh, close to the patella (lateral bulging). Figure 25: The patient underwent a biopsy in another hospital, using an inadequate horizontal incision.
Figura 26: Cintilografia com hipercaptação nos tecidos moles.
Figura 27: Rm mostra a cortical do fêmur preservada.
Figure 26: Scintigraphy with high uptake in soft tissues. Figure 27: MRI shows the preserved femoral cortex.
Figura 28: RM de Condrossarcoma extra ósseo. Cortes axiais,com lesão apresentando baixo sinal em T1, acometendo a fascia lata.
Figura 29: Em T2 apresenta alto sinal, heterogênio, com evidente infiltração e destruição da fascia.
Figure 28: MRI of extraosseous chondrosarcoma. Axial sections, with a lesion showing low signal on T1, affecting the fascia lata. Figure 29: On T2, it shows high signal, heterogeneity, with evident infiltration and destruction of the fascia.
Figura 30: Em T1 com supressão verificamos alto sinal.
Figura 31: Captação intensa de contraste.
Figure 30: In T1 with suppression we see high signal. Figure 31: Intense contrast capture.

  Regarding  HISTOLOGY  , they present different aspects, which can be separated into:
A) Degree of Anaplasia : they are classified into  grades I ,
II  and  III , depending on cellularity and atypical mitoses11,24, being:
-Chondrosarcoma grade I , well differentiated, similar structure to hyaline cartilage but with increased cellularity, irregular distribution of gaps and a varied number of cells per gap, sometimes binucleate, hyperstained and polymorphic (Figure 10.32);
-Grade II chondrosarcoma , moderately differentiated, greater number of cells due to gaps with great polymorphism and cellular disarray (Figure 10.33);

– Grade III chondrosarcoma , undifferentiated, marked anaplasia, myxoid areas, undifferentiated cells and scarce hyaline matrix (Figure 34).

B) Dedifferentiated : clear areas of mature cartilage and other areas with immaturity, showing high cellularity and atypical mitoses 8  (Figure 35);

C) Mesenchymal : rare, presenting fields of hyaline cartilage interspersed with undifferentiated neoplasia of small, round cells 8  (Figure 36), and

D) Clear Cells : rare, presents round cells with clear or vacuolated cytoplasm and little cellular atypia 2   Figure 37).

Figura 32: Condrossarcoma grau I, que pode apresentar dificuldade no diagnóstico diferencial histológico com o condroma.
Figure 32: Grade I chondrosarcoma, which may present difficulty in the histological differential diagnosis with chondroma.
Figura 33: Condrossarcoma grau II, maior número de mitoses e desordem.
Figura 34: Condrossarcoma grau III, maior celularidade e atipia.
Figure 33: Grade II chondrosarcoma, greater number of mitoses and disorder. Figure 34: Grade III chondrosarcoma, greater cellularity and atypia.
Figura 35: Condrossarcoma desdiferenciado.
Figura 36: Condrossarcoma mesenquimal.
Figure 35: Dedifferentiated chondrosarcoma. Figure 36: Mesenchymal chondrosarcoma.
Figura 37: Condrossarcoma de células claras.
Figure 37: Clear cell chondrosarcoma.

As for  ORIGIN,  they can be:

1-  Primary , when it occurs in tissue that had no previous injury (Figures 38 and 39).

2-  Secondary , which originates over a pre-existing benign cartilaginous lesion 2,3,6,8,13,14,23,24  (Figures 40 to 44).

Figura 38: Radiografia de condrossarcoma primário no anel obturador da pelve direita.
Figure 38: Radiograph of primary chondrosarcoma in the obturator ring of the right pelvis.
Figura 39: Tomografia da lesão.
Figure 39: Tomography of the lesion.
Figura 40: Observe o aspecto do quadril esquerdo deste paciente, portador de Ollier, aos três anos de idade.
Figure 40: Observe the appearance of the left hip of this patient, with Ollier, at three years of age.
Figura 41: Paciente com 14 anos.
Figura 42: Após seis anos a lesão cartilaginosa da região trocanteriana apresentou degeneração sarcomatosa, condrossarcoma secundário.
Figure 41: 14-year-old patient. Figure 42: After six years, the cartilaginous lesion in the trochanteric region showed sarcomatous degeneration, secondary chondrosarcoma.
Figura 43: Aos vinte anos, paciente com encondromatose múltipla, unilateral (doença de Ollier). A lesão do quadril degenerou-se para condrossarcoma.
Figure 43: At twenty years old, patient with multiple, unilateral enchondromatosis (Ollier's disease). The hip lesion degenerated into chondrosarcoma.
Figura 44: Ressecado o 1/3 proximal do fêmur com a musculatura glútea (com margem). Reconstruido com prótese total do quadril constrita.
Figure 44: The proximal 1/3 of the femur with the gluteal muscles was resected (with margin). Reconstructed with constricted total hip prosthesis.

Secondary chondrosarcoma occurs in Ollier’s disease or Maffucci Syndrome in 20 to 30% of cases 2,29 , and can also occur as a result of a single enchondroma, although it is rare in this situation.

   It can also develop from the cartilaginous layer of a solitary osteochondroma, less than 1%, or multiple osteochondromatosis, around 10% 2  and more rarely secondary to Paget’s disease.

   In osteochondroma, when an increase in the lesion is observed after skeletal maturity, the possibility of malignancy must be considered.

   This malignancy is characterized by an increase in the thickness of the cartilaginous layer greater than 2 cm29, irregular calcifications, the appearance of pain and a heterogeneous appearance of the lesion2,6 .

   Due to the different morphological characteristics and clinical behavior of the chondrosarcoma subtypes, we consider it educational to discuss individually the incidence, clinical, radiographic and anatomopathological aspects of each subtype:

1. Central or Conventional Chondrosarcoma

   It is the most frequent of chondrosarcomas, accounting for 90%8. It represents between 10.0 and 14.5% of all primary malignant bone tumors 2,23 .

   According to Dahlin and Jaffe, it affects men and women equally, while for other authors there is a male prevalence that varies from 10% 6,9,14,29  to around 70%, according to Schajowicz 2 .

   It occurs more frequently in adults between 30 and 60 years old 2,14,24,29.

   Its location is most frequent in the proximal segment of the femur, humerus and tibia; being rare in short bones 2,23 .

   Pain can be an insidious symptom for several years, evolving with slow growth, increased volume, restricted mobility, with the skin sometimes becoming red and hot 23 . As it is oligosymptomatic, pathological bone fractures are often the first manifestation of the disease 2,24.

   The radiograph shows a radio-transparent metaphyseal lesion, replacing the bone marrow. The tumor grows towards the epiphysis or diaphysis and erodes the inner cortex, causing punch-hole lesions. Expansion of the medullary portion of the bone may occur, with cortical inflation (Figures 45 and 46).

Figura 45: Lesão radiotransparente, erosão da cortical interna, insuflação e expansão da porção medular do osso com espessamento da cortical medial.
Figure 45: Radiolucent lesion, erosion of the inner cortex, inflation and expansion of the medullary portion of the bone with thickening of the medial cortex.
Figura 46: coloração branco-azulada, focos de calcificação e lóbulos. Neste caso houve erosão da cortical e extravazamento do tumor.
Figure 46: bluish-white color, calcification foci and lobes. In this case, there was erosion of the cortex and extravasation of the tumor.

   The x-ray shows frequent calcifications (Figure 47). This results from the neoangiogenesis of cartilaginous tissue, which degenerates. This process is accelerated in chondrosarcomas and slowed in benign and low-grade cartilaginous lesions. Calcifications can be speckled, cotton flaked or ring-shaped 23,2,24,13,6,29,5 .

   Bone mapping helps in tumor staging (Figure 48). Magnetic resonance imaging and tomography are important for evaluating the intramedullary extension and extraosseous involvement of the lesion 2 .

   Macroscopy shows a bluish-white color with foci of yellowish calcification, forming lobes separated by connective tissue septa and areas of necrosis 2,24,6,14  (Figures 49 and 50).

Figura 47: Lesão radiotransparente, com erosão da cortical interna em saca bocado, insuflação, expansão da porção medular do osso e focos de calcificação salpicados.
Figure 47: Radiolucent lesion, with erosion of the inner cortex in a piece-like manner, insufflation, expansion of the medullary portion of the bone and speckled foci of calcification.
Figura 48: Lesão única, hipercaptante no fêmur direito.
Figure 48: Single, hypercapsulating lesion on the right femur.
Figura 49: foto de segmento da diáfise femoral. A cirurgia com ressecção oncológica é o procedimento mais importante para a cura deste tumor.
Figure 49: photo of a segment of the femoral shaft. Surgery with oncological resection is the most important procedure for curing this tumor.
Figura 50: aspecto macroscópico do corte da peça. Verificamos focos de calcificação, espessamento e erosão em saca bocado da cortical interna.
Figure 50: Macroscopic appearance of the part cut. We verified foci of calcification, thickening and erosion in a small portion of the inner cortex.

   Microscopically, central chondrosarcoma presents hypercellularity, bulky nuclei, sometimes binucleate, polymorphism, atypia, myxoid intercellular matrix, invasion and destruction of adjacent bone trabeculae. This histology may also present a low or high degree of dedifferentiation.

   Low-grade lesions can be confused with benign cartilaginous tumors, as the histological difference between these tumors is subtle. A low-grade lesion is considered when there is moderate cellularity, atypia, polymorphism and binucleate forms 12  (Figure 51).

   At high levels, there is hypercellularity, atypia and marked polymorphism, with several mitotic figures in the myxoid intercellular matrix 14,12 .

Figura 51: Histologia do caso anterior com grau moderado de atipia celular. Condrossarcoma grau I / II.
Figure 51: Histology of the previous case with a moderate degree of cellular atypia. Grade I/II chondrosarcoma.

 2. Juxtacortical (or surface) chondrosarcoma
   Also called parosteal or periosteal, these tumors develop on the surface of the bone.
   They are rare, representing 20% ​​of chondrosarcomas, in general, they are low grade and occur in young adults 23 . They affect the metadiaphyseal region of long bones, with preference in the posterior and distal region of the femur, anterior and proximal tibia and proximal humerus 14 .

   The lesion involves the cortex, with poorly defined margins and grows more quickly than conventional chondrosarcoma. They have a hard consistency, are generally painless and without signs of inflammation on the underlying skin 23  (Figures 52 to 60).

   In the highly malignant variable, there is cortical erosion and the presence of a palpable, soft and painful tumor 2 .

Figura 52: Radiografia da coxa direita, com lesão justacortical na face medial da diáfise femoral.
Figure 52: Radiograph of the right thigh, with juxtacortical lesion on the medial surface of the femoral shaft.
Figura 53: Tomografia mostrando lesão justacortical, sem comprometimento do canal medular.
Figure 53: Tomography showing juxtacortical lesion, without involvement of the spinal canal.
Figura 54: Tomografia com densidade para tecidos moles, evidenciando calcificações na musculatura da coxa e tumor heterogênio.
Figure 54: Soft tissue density tomography, showing calcifications in the thigh muscles and a heterogeneous tumor.
Figura 55: Tomografia com densidade para osso, com tumor extracortical envolvendo a superfície do fêmur, na face lateral, anterior e medial.
Figure 55: Density tomography for bone, with extracortical tumor involving the surface of the femur, on the lateral, anterior and medial aspects.
Figura 56: Corte da peça cirúrgica com o condrossarcoma justacortical, apenas na superfície do osso. Observe que a medular óssea e a cortical interna apresentam aspecto normal.
Figure 56: Section of the surgical specimen with juxtacortical chondrosarcoma, only on the surface of the bone. Note that the bone marrow and inner cortex appear normal.
Figura 57: Condrossarcoma justacortical da extremidade distal do fêmur.
Figura 58: RM em T1 verificamos o comprometimento anterior, lateral e posterior extracortical
Figure 57: Juxtacortical chondrosarcoma of the distal end of the femur. Figure 58: MRI in T1 we verified the anterior, lateral and posterior extracortical involvement
Figura 59: corte da peça mostrando o envolvimento da superfície do osso, sem invasão da medular. Figura 60: RM com forte captação de contraste.
Figura 60: RM com forte captação de contraste.
Figure 59: section of the piece showing involvement of the bone surface, without invasion of the medullary bone. Figure 60: MRI with strong contrast uptake.

  Radiographically, a transparent bone rarefaction lesion may occur, with foci of calcification between the eroded bone cortex and the elevated periosteum.

Macroscopically, at diagnosis, it is a lesion larger than five centimeters, lobulated and adhered to the surface of the bone.

Microscopy is similar to conventional chondrosarcoma. Tumor nodules may invade peripheral soft tissues.


3.  Peripheral  or  exophytic chondrosarcoma  It differs from the juxtacortical type, as it arises from a pre-existing osteochondroma. Malignancy of an osteochondroma should be considered when the lesion grows after skeletal maturity, without previous trauma or repetitive friction.

   Radiographically, they present large-volume tumors, with radiopaque areas on the periphery, with a globose or ovoid appearance, smooth or multilobulated surface, calcified in the central portion, strongly implanted in the host bone, appearing to be a vegetative tumor, which is not always confirmed, as it has limits well-defined external elements 23 .

   They grow more slowly than the central type and are often large at diagnosis 12,14.
   This type of chondrosarcoma can only be cured with adequate resection. A parietal resection of the bone portion where the osteochondroma is located must be performed 29,12 , avoiding blunt dissection of the surface of the lesion.

   In sarcomatous degeneration, there is an increase in the thickness of the cartilage layer, with irregular calcifications and pain where previously it was painless 2,4 .
   They are most common in the ilium and scapula, followed by the proximal region of the femur, distal femur, proximal humerus and proximal tibia 3,12,23,29.
   Figures 61 to 103 illustrate examples of peripheral chondrosarcoma, secondary to osteochondroma, with different aspects, in the main frequency locations.

Figura 61: Condrossarcoma secundário à osteocondroma. Na TC observamos o aspecto característico da exostose (cortical do osso continuando-se com a cortical da lesão e a medular do osso com a medular da lesão).
Figure 61: Chondrosarcoma secondary to osteochondroma. On CT we observed the characteristic appearance of exostosis (cortical of the bone continuing with the cortical of the lesion and the medullary of the bone with the medullary of the lesion).
Figura 62: Na cintilografia a captação mostra atividade da lesão, após a maturidade esquelética.
Figure 62: On scintigraphy, the capture shows activity of the lesion, after skeletal maturity.
Figura 63: Queixa de dor e aumento da lesão na radiografia.
Figure 63: Complaint of pain and enlargement of the lesion on the x-ray.
Figura 64: A Ressonância em T1 é heterogênea e há grande captação de contraste.
Figura 65: A biópsia nem sempre é positiva e deve-se operar com margem oncológica, pois há áreas de osteocondroma e áreas de condrossarcoma.
Figure 64: The T1 MRI is heterogeneous and there is great contrast uptake. Figure 65: The biopsy is not always positive and it must be operated with an oncological margin, as there are areas of osteochondroma and areas of chondrosarcoma
Figura 66: Na osteocondromatose a incidência de degeneração é dez vezes maior e é mais frequênte quando a lesão é na raíz do membro. O mapeamento mostra captação do lado esquerdo, no ilíaco e na perna.
Figure 66: In osteochondromatosis the incidence of degeneration is ten times greater and is more frequent when the injury is at the root of the limb. The mapping shows uptake on the left side, in the iliac bone and in the leg.
Figura 67: Na hemipelve esquerda trata-se de condrossarcoma.
Figura 68: A radiografia exibe a lesão da osteocondromatose na perna.
Figure 67: In the left hemipelvis, it is chondrosarcoma. Figure 68: The x-ray shows the osteochondromatosis lesion on the leg.
Figura 69: TC destaca a agressividade da lesão do iliaco esquerdo. Figura 70: Na RM em T1 vemos áreas de baixo sinal e áreas de sinal intermediário.
Figura 70: Na RM em T1 vemos áreas de baixo sinal e áreas de sinal intermediário.
Figure 69: CT highlights the aggressiveness of the left iliac injury. Figure 70: On T1 MRI we see areas of low signal and areas of intermediate signal.
Figura 71: Em T2 sem supressão vemos áreas de alto sinal e de sinal intermediário.
Figura 72: T1 com supressão revela que a lesão é heterogenea.
Figure 71: In T2 without suppression we see areas of high signal and intermediate signal. Figure 72: T1 with suppression reveals that the lesion is heterogeneous.
Figura 73: Em T1, com supressão e contraste, verificamos a captação em um tumor sólido.
Figura 74: Em FFE destaca-se alguns focos de calcificação.
Figure 73: On T1, with suppression and contrast, we verified uptake in a solid tumor. Figure 74: In FFE some foci of calcification stand out.
Figura 75: Hipercaptação na cintilografia óssea, sugerindo aumento de atividade metabólica.
Figura 76: A arteriografia revela hipervascularização.
Figure 75: Hyperuptake on bone scintigraphy, suggesting increased metabolic activity. Figure 76: Arteriography reveals hypervascularization.
Figura 77: Aspecto cirúrgico do tumor, que deve ser ressecado com margem oncológica.
Figura 78: Radiografia do pós operatório imediato.
Figure 77: Surgical aspect of the tumor, which must be resected with an oncological margin. Figure 78: Radiograph of the immediate post-operative period.
Figura 79: Rx da peça
Figura 80: Comparando com o corte anatômico verificamos a correspondência das calcificações e da grande capa de cartilagem.
Figure 79: X-ray of the piece Figure 80: Comparing with the anatomical section, we check the correspondence of the calcifications and the large cartilage layer.
Figura 81: Osteocondromatose em paciente adulto, com aumento de volume da coxa esquerda. Discreto aumento da temperatura local e dor.
Figure 81: Osteochondromatosis in an adult patient, with increased volume of the left thigh. Mild increase in local temperature and pain.
Figura 82: Radiografia do fêmur esquerdo com volumoso osteocondroma séssil, com a superfície externa borrada, sem contornos nítidos.
Figure 82: Radiograph of the left femur with a large sessile osteochondroma, with the external surface blurred, without clear contours.
Figura 83: TC com janela para osso.
Figura 84: TC com janela para tecidos moles.
Figure 83: CT with bone window. Figure 84: CT with soft tissue window.
Figura 85: Tumor na face medial, áreas de baixo sinal.
Figura 86: Calcificação, heterogenicidade e intensa captação na periferia e no interior da lesão.
Figure 85: Tumor on the medial face, areas of low signal. Figure 86: Calcification, heterogenicity and intense uptake in the periphery and interior of the lesion.
Figura 87: Lesão na região metadiafisária proximal da tíbia, condensante, com bordas irregulares e focos de calcificação com aspecto algodonoso.
Figure 87: Lesion in the proximal metadiaphyseal region of the tibia, condensing, with irregular edges and foci of calcification with a cotton-wool appearance.
Figura 88: Radiografia mostrando continuidade da lesão com o osso e deformidade da cortical posterior, sugerindo degeneração de osteocondroma.
Figure 88: Radiograph showing continuity of the lesion with the bone and deformity of the posterior cortex, suggesting osteochondroma degeneration.
Figura 89: Aspecto clínico.
Figura 90: Tomografia caracterizando o osteocondroma pré-existente e a lesão irregular da degeneração sarcomatosa.
Figure 89: Clinical appearance. Figure 90: Tomography characterizing the pre-existing osteochondroma and the irregular lesion of sarcomatous degeneration.
Figura 91: RM em T1 com áreas de baixo sinal e sinal intermediário.
Figura 92: RM em T2 com aspecto heterogênio e alto sinal.
Figure 91: T1 MRI with areas of low signal and intermediate signal. Figure 92: T2 MRI with heterogeneous appearance and high signal.
Figura 93: T1 com supressão e contraste caracterizando a agressividade da lesão. Áreas de baixo sinal com focos de calcificação, outras de sinal intermediário e alto sinal. Forte captação de contraste, principalmente na periferia do tumor. Isto é caracteristico neste tipo de condrossarcoma por aumento da capa cartilaginosa.
Figure 93: T1 with suppression and contrast characterizing the aggressiveness of the lesion. Areas of low signal with foci of calcification, others of intermediate signal and high signal. Strong contrast uptake, especially at the periphery of the tumor. This is characteristic of this type of chondrosarcoma due to an increase in the cartilaginous layer.
Figura 94: RM coronal T1 mostrando lesão heterogênea, exofítica, com bordas irregulares. A biópsia pode mostrar apenas áreas de osteocondroma.
Figure 94: Coronal T1 MRI showing a heterogeneous, exophytic lesion with irregular edges. The biopsy may only show areas of osteochondroma.
Figura 95: Nestes casos devemos tratar sempre como condrossarcoma e ressecar o tumor com margem.
Figura 96: Deve-se ressecar o tumor com margem, principalmente na superfície.
Figure 95: In these cases we should always treat it as chondrosarcoma and resect the tumor with a margin. Figure 96: The tumor must be resected with a margin, especially on the surface.
Figura 97: A exposição deve ser ampla, sem abrir a fina camada de tecido frouxo que recobre a lesão, procurando-se evitar a recidiva local. A degeneração sarcomatosa é mais frequênte na superfície da lesão.
Figure 97: Exposure must be broad, without opening the thin layer of loose tissue that covers the lesion, trying to avoid local recurrence. Sarcomatous degeneration is most common on the surface of the lesion.
Figura 98: Aspecto após a ressecção parietal do tumor, removendo uma faixa de tecido ósseo sadio na profundidade da lesão. Observe o aspecto macroscópico normal do leito cirúrgico.
Figure 98: Appearance after parietal resection of the tumor, removing a strip of healthy bone tissue in the depth of the lesion. Observe the normal macroscopic appearance of the surgical bed.
Figura 99: Peça ressecada em bloco.
Figura 100: Radiografia da peça cirúrgica realçando a heterogeneidade da lesão.
Figure 99: Piece dried in block. Figure 100: Radiograph of the surgical specimen highlighting the heterogeneity of the lesion.
Figura 101: Radiografia pós operatória em perfil, com bom aspecto, sem sinais de recorrência.
Figure 101: Post-operative radiograph in profile, with good appearance, without signs of recurrence.
Figura 102: Radiografia mostrando cicatrização do leito ósseo cruento, que se encontra em remodelação.
Figure 102: Radiograph showing healing of the raw bone bed, which is undergoing remodeling.
Figura 103: Paciente curado do condrossarcoma periférico, exofítico, que cresceu sobre um osteocondroma. Função normal do membro operado.
Figure 103: Patient cured of peripheral, exophytic chondrosarcoma, which grew over an osteochondroma. Normal function of the operated limb.

 The histological diagnosis of well-differentiated chondrosarcoma is challenging.

   The same histological appearance of irregularity in the arrangement and number of cells within the chondroid matrix, with nuclear changes of hyperchromasia, discrete polymorphism and some atypical mitoses, can represent different entities: chondrosarcoma, when located in the roots of limbs, and chondroma when found in the hands and feet.
   Under microscopy, the description of chondromas of the hands and feet is similar to that of central chondrosarcoma 23 .
   Data on clinical history, location and imaging aspects must be valued to conclude the diagnosis and define appropriate management 12,14,23.

4. Mesenchymal Chondrosarcoma

   Initially described by Lichtenstein and Bernstein in 1959 16 , it is extremely rare 24 . The largest case series, thirty cases, was published by Salvador 18 .

   It is characterized by areas of differentiated cartilage, interspersed with mesenchymal tissue with round or fusiform cells, highly vascularized with a hemangiopericytic pattern 2  (Figure 104).

   It presents a slight predominance in females, occurs between the second and third decades of life 2,6,14 , and frequently appears in the lower extremities, jaws, mandible and ribs. Pain and increased volume are the main clinical symptoms 2.

   It may resemble conventional chondrosarcoma.
   Radiographically, it has an aggressive appearance 29  with involvement of soft tissues and frequent pulmonary metastases 12.

   It presents a high degree of malignancy, with small round or fusiform blue cells surrounding cartilage islands, with a well-differentiated benign appearance, a pattern similar to hemangiopericytoma 24,29 .

   The cells resemble undifferentiated chondroblasts 6 .

   Macroscopically, they present areas of grayish-white or yellowish color, with a soft consistency, alternating with hardened areas, with a cartilaginous appearance and foci of calcification.

Figura 104: Histologia de condrossarcoma mesenquimal. Áreas de cartilagem diferenciada, entremeada por células redondas e fusiformes, com vasos de padrão hemangioperiocítico, encontrado no tecido mesenquimatoso.
Figure 104: Histology of mesenchymal chondrosarcoma. Areas of differentiated cartilage, interspersed with round and fusiform cells, with vessels with a hemangioperiocytic pattern, found in the mesenchymal tissue.

5. Clear Cell Chondrosarcoma

   Rare neoplasm, located in the epiphyses of long bones and composed of round cells with abundant, clear cytoplasm and numerous giant cells 2,24 ,  29  (Figure 105).

   It essentially affects adults and involves the proximal femur, humerus and tibia 2,29 .

   On radiography, it appears as a well-defined epiphyseal lesion, similar to a giant cellular tumor (GCT) 2  or chondroblastoma, interpreted by some authors as the malignant evolution of this neoplasm 4 .

   Diagnostic doubts regarding chondroma may occur, and radiographic aggressiveness must always be considered to define appropriate management.

Figura 105: Histologia de condrossarcoma de células claras. Células redondas, com citoplasma claro e células gigantes.
Figure 105: Histology of clear cell chondrosarcoma. Round cells, with clear cytoplasm and giant cells.

6. Dedifferentiated Chondrosarcoma

   It is a highly anaplastic sarcoma together with a low-grade malignant chondrosarcoma, presenting an abrupt transition between the two 29  (Figure 106).

   It may have the appearance of malignant fibrous histiocytoma, fibrosarcoma, osteosarcoma or rhabdomyosarcoma 2,29 .

   It affects the pelvis and long bones, particularly the femur and humerus. It occurs in patients over twenty years of age and has a peak incidence between 40 and 50 years of age, with no predilection between men and women.

   On radiography, the lesions are similar to common chondrosarcoma, but the presence of a larger area of ​​cortical lysis suggests an aggressive lesion 29 .

Figura 106: Histologia de condrossarcoma desdiferenciado. Áreas nítidas de cartilagem madura, com áreas de imaturidade, caracterizada por celularidade alta e mitoses atípicas.
Figure 106: Histology of dedifferentiated chondrosarcoma. Clear areas of mature cartilage, with areas of immaturity, characterized by high cellularity and atypical mitoses.

Differential diagnosis:

   It presents a differential diagnosis with myositis ossificans, chondromyxoid fibroma, GCT, non-Hodgkin lymphoma 6,23,29  and aneurysmal bone cyst, due to its multiloculated nature. Histologically, the juxtacortical subtype resembles chondroma, osteochondroma, chondroblastoma and surface osteosarcoma 16 .

   Clear cell chondrosarcoma has malignant chondrocytes with clear cytoplasm, osteoclast-like giant cells, and intralesional reactive bone formation causing confusion with osteosarcoma.

   Mesenchymal chondrosarcoma is formed by islands of well-differentiated hyaline cartilage surrounded by sheets of small, round cells, reminiscent of hemangiopericytoma and Ewing’s sarcoma  14.

   Central chondroma of long bones, chondrosarcoma and bone infarction are often difficult to differentiate, requiring clinical and radiographic monitoring to assess whether or not the lesion has progressed, before defining the course of action. Biopsy
is often not definitive for diagnosis 12,23,29.

Treatment:

   The treatment of chondrosarcoma is surgical 25 , and a wide resection must be chosen, including the biopsy path 13,21.

    Radiotherapy is ineffective 6  in controlling this neoplasm. For high-grade lesions, it is possible to discuss the indication of chemotherapy using the protocol for large cell sarcomas, based on anthracyclines 9999.  For mesenchymal chondrosarcoma, which presents a predominance of small and undifferentiated cells, chemotherapy, when indicated, falls under the protocol of treatment of Ewing Tumor. 888

   In both cases, the response to chemotherapy is usually poor 6 . The treatment of this neoplasm must be individualized for each clinical subtype:

– Central chondrosarcoma  has high cure rates with appropriate surgery, therefore its treatment with intralesional curettage cannot be underestimated, even followed by complementary adjuvant methods, whether with phenol, liquid nitrogen, electrothermia or CO 2  laser 21.

   Therefore, in cases of diagnostic doubt between chondroma and grade I chondrosarcoma, it is preferable to observe the evolution of this lesion, as it is known that the biopsy will not be conclusive, as the histological differential diagnosis between chondroma and grade I chondrosarcoma is difficult.

   In some cases, these lesions can be treated with conservative surgery without performing a prior biopsy 21 .

   When imaging tests: radiography, tomography and magnetic resonance, show a central lesion, without erosion of the internal cortex, with a casual and painless finding, it should be reevaluated initially within three months, if unchanged, repeated within six months and If the lesion remains unchanged, annual reassessments are scheduled.

   If, at any time, there is a change in the clinical picture or imaging, it should be treated as central chondrosarcoma, carrying out wide resection of the lesion and reconstruction with non-conventional endoprosthesis, osteosynthesis with autologous or homologous graft or ablative surgery as necessary. of each case.

   In the experience of these authors, it is unnecessary to operate on a painless chondroma when it is found casually, without radiographic aggressive characteristics. Performing an intralesional curettage, with local adjuvant and graft or cement, will not eliminate the need for careful observation. If the anatomopathological examination of the entire curettage reveals that it was chondrosarcoma, it will be much worse to re-operate on this region that has already been surgically manipulated.

   There are several cases of “chondroma” in which the histology of intralesional curettage corroborated the biopsy appearance of “chondroma” and however had an unfavorable outcome. When monitoring these patients, imaging tests revealed that there was a “new” lesion at the site and that it was now chondrosarcoma.

   In these curettages, local and distant dissemination and even dedifferentiation of the chondrosarcoma may occur, significantly worsening the prognosis.

–  Juxtacortical chondrosarcoma, treatment is essentially surgical, with partial parietal resection EXAMPLE  possible   when possible, an effective procedure with lower morbidity compared to segmental resection.

– Peripheral chondrosarcoma , secondary to osteochondroma, care must be taken especially with the surface of the lesion, which presents anaplasia.

   The surrounding soft tissue perimysium should be removed as an oncological margin to prevent local recurrence.

   It is important to highlight that when there is growth of a bony exostosis after skeletal maturity, heterogeneous calcification, thick cartilaginous cap, unrelated to friction or trauma, it is probably a chondrosarcoma.

   In this situation, a negative biopsy sample does not exclude the possibility of malignancy in the remainder of the lesion, and resection surgery with an oncological margin must be performed, paying special attention to the surface of the lesion.

– Mesenchymal chondrosarcoma , in addition to the need for local control with extensive surgery, may eventually be indicated for additional chemotherapy treatment 9999 .

– Dedifferentiated Chondrosarcoma , such as Clear Cell Chondrosarcoma, local control must be carried out with extensive surgery and chemotherapy with cisplatin and doxorubicin 9999.

Complications:
Intralesional curettage of chondrosarcoma can lead to local recurrence of the disease and more aggressive histological dedifferentiation.

   In cases of dedifferentiated chondrosarcomas, hematogenous metastases to the lungs are frequent, which may present lymphatic dissemination and local recurrence 29 . Many chondrosarcomas tend to spread locally 14 , reaching enormous sizes and becoming inoperable, causing death due to compression or complications from this local spread.

   Local recurrence increases the incidence of lung metastases 21.

Bibliography

1. ACKERMAN, L.V.; SPJUT, HJ Tumors of bone and cartilage. Atlas of tumor pathology. Washington, Air Force Inst. Pathology, 1962, fasc, 4.
2. CANALE, ST Campbell.Barueri orthopedic surgery: Manole; 2006
3. DAHLIN, DC Bone tumors. Barcelona: Ediciones Toray S/A; 1982
4. DORFMAN, HD; CZERNIAK, B. Bone tumors. St Louis, CV Mosby Co., 1997, chap. 7, p.410.
5. EDEIKEN, J.; HODES, PJ Radiological diagnosis of human illnesses. Buenos Aires, Panamericana, 1977, chap. 15.
6. ETCHEBEHERE, M. Malignant cartilaginous tumors: Chondrosarcomas. In: Camargo OP Clínica Ortopédica. Rio de Janeiro: Med si; 2002. p. 753-759
7. FELDMAN, F. Cartilaginous tumors and cartilage-forming tumor like conditions of the caps and soft tissues. In: Diseases of the Skeleton System (Roentgen Diagnosis). Part. 6 – Bone Tumors, New York, Springer-Verlag, 1977, p.177.

8. FLETCHER, CDM, Unni KK, WHO – Merters F. (Eds.): World Health Organization. Classification of Tumors. Pathology and Genetics of Tumors of Soft Tissue and Bone. IARC Press: Lyon 2002.

9. GREENSPAN, A. Orthopedic radiology. Rio de Janeiro: Guanabara; 2001. 
10. HENDERSON, ED; Le PAGE, GA Apud FELDAMAN, F. Cartilaginius tumors and cartilage forming tumor like conditions of the bone and soft tissues. In: Disease of the Skeletal System (Roentgen Diagnosis).
Part. 6 – Bone tumors, New York, Springer Verlag, 1977, p.182.
11. HUVOS, AG Bone tumors Diagnosis, Treatment and Prognosis. Philadelphia, WB Saunders Co., 1979, p. 13.
12. JAFFE, HL Tumors and tumoral states of bones and joints. Mexico: La Prensa Medica Mexicana;1966.

13. JESUS-GARCIA, R. – Reynaldo Jesus-Garcia
14. LICHTENSTEIN, L. Barcelona: Talleres Graphics Ibero-Americanos; 1975.
15. LICHTESTEIN, L. Bone Tumor. 4 Ed St. Louis, CV Mosby Co., 1972, chap. 15.
16. LICHTESTEIN, L.; BERNSTEIN, D. Unusual benign and malignant chondroid tumors of bone. Cancer, 12:1142, 1959.
17. MARCOVE, RC Chondrosarcoma: Diagnosis and treatment. In: Orthopedic Clinics of North America. Tumors of the musculoskeletal apparatus. Buenos Aires, Panamericana, 1977, chap. 7.
18. MARCOVE, RC et al. Chondrosarcoma of the pelvis and upper end of the femur. In the analysis of factors influencing survival time in 113 cases. J. Bone Joint Surg., 54A:61, 1972.

19. MARCOVE, RC; SHOJI, H.; HARLEN, M. Altered carbohydrate metabolism in cartilaginous tumors. Contemp. Surg. 5:53, 1974.
20. McFARLAND, GBJr.; McKINLEY, L.M.; REED, RJ Dedifferentiation of low grade chondrosarcomas. Clinic. Orthop., 122:157, 1971.
21. MENENDEZ, LR Orthopedic knowledge update: Updates in orthopedic surgery and traumatology. Barcelona: Ars Medica; 2003.
22. O’NEAL, LW; ACKERMAN, LV Chondrosarcoma of cap. Cancer, 5:551, 1952.
23. PROSPERO, JD Bone Tumors. São Paulo, Roca, 2001, chap. II.
24. ROBBINS. Structural and functional pathology. Rio de Janeiro: Guanabara; 1996.

25. ROMSDAHL, M.; EVANS, H.L.; AYALA, AG Surgical treatment of chondrosarcoma. In: Management of primary bone and soft tissue tumors. Chicago, Year book med. Publisher Inc., 1977, p. 125.

26. ROMSDAHL, M.; Evans, H.L.; Ayala, AG Surgical treatment of chondrosarcoma. In: Management of primary bone and soft tissue tumors. Chicago. Year book med. Publisher Inc., 1977, p.125.
27. SAVIOR, AH; BEABOUT, JW; DAHLIN, DC Mesenchymal chondrosarcoma. Cancer, 28:605, 1971.
28. SCHAJOWICZ, F. Juxtacortical Chondrosarcoma. J. Bone Joint. Surg., 59B:473, 1977.
29. SCHAJOWICZ, F. Tumors y Lesiones Seudotumorales de Huesos y Articulaciones. Buenos Aires: Editora Médica Panamericana; 1982.

30. TORNBERG, DN; RICE, R.W.; JOHNSTON, AD The ultrastructure of chondromyxoid fibroma.Clin. Orthop. Rel. Research, 95:295, 1973.
999. J Clin Oncol 30:abstract 100:23,2012(maluf)
888. Buzaide, AC; Maluf, FC; Rocha Lima, CM
Brazilian Clinical Oncology Manual. Dendrix Edition and Design ltda. São Paulo (XI) Adult Bone Sarcomas, 560-79. 2013

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

Phone: +55 11 3231-4638  Cell:+55  11 99863-5577   Email:  drpprb@gmail.com

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Chondroblastoma

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Chondroblastoma is a benign cartilage-forming neoplasm corresponding to approximately 1.8% of bone tumors.

Chondroblastoma

Condroblastoma da cabeça femoral – Lesão intra-articular – Pós operatório de 1 ano – Pós operatório de 8 anos
Chondroblastoma of the femoral head – Intra-articular lesion – 1 year after surgery – 8 years after surgery
Metástases Pulmonares assintomáticas.
Asymptomatic lung metastases.

Codman, in 1931, described it as a different form of manifestation of the “calcified giant cell tumor” of the proximal humerus. It was later found to be a tumor other than the gigantocellular tumor (GCT).

It preferentially affects the epiphysis of long bones, as a bone rarefaction lesion, with foci of calcification, in male patients, in the first and second decade of life, therefore with the growth plate open. Therefore, it affects the epiphyseal region of growing long bones (figs. 1 and 2) and, less frequently, the apophyseal portion (figs. 3 and 4).

Figura 1 condroblastoma epifisário do fêmur.
Figure 1 Epiphyseal chondroblastoma of the femur.
Figura 2 erosão da cartilagem articular.
Figure 2 erosion of articular cartilage.
Figura 3 condroblastoma da apófise do grande trocânter. Figura 4 – aumento significativo da lesão, com fratura arrancamento do grande trocânter.
Figure 3 Chondroblastoma of the apophysis of the greater trochanter. Figure 4 – significant increase in the injury, with tearing fracture of the greater trochanter.

This injury, as it occurs intra-articularly, can present a clinical picture similar to arthritis, when it causes reabsorption of the epiphysis (or apophysis), erosion of the cortical bone and joint invasion, leading to arthralgia, which can cause deformity and fracture and joint subsidence. It can, therefore, present local aggressiveness such as cortical erosion, growth plate erosion and joint invasion.

When there is radiographic manifestation of local aggressiveness, it is generally associated with areas of aneurysmal bone cyst. It presents differential diagnosis with simple bone cyst, aneurysmal, osteomyelitis, tuberculosis, arthritis, chondroma, giant cell tumor (osteoclastoma), early osteoid osteoma, pseudo-tumorous lesions, among others.

The treatment of chondroblastoma is surgical and must be carried out as soon as possible, as these lesions, despite being histologically benign, quickly progress to destruction of the local bone framework. It consists of intralesional curettage followed by local adjuvant (phenol, electrothermia, liquid nitrogen, etc.) and placement of a bone graft or cement (polymethylmethacrylate). 

In very advanced lesions, segmental resection followed by placement of a prosthesis or arthrodesis may be necessary. The best indication to avoid local recurrence is segmental resection.

However, due to the articular location of the chondroblastoma, it is preferable to provide adequate surgical access to each region, as in this example, which affects the posteromedial region of the femoral head (fig. 5 and 6).

Fig. 5- Condroblastoma da região medial da cabeça femoral direita.
Fig. 5- Chondroblastoma of the medial region of the right femoral head.
Fig. 6 – Lesão lítica posterior e medial na cabeça femoral direita.
Fig. 6 – Posterior and medial lytic lesion on the right femoral head.
We perform careful intra-lesional curettage, followed by local adjuvant, such as phenol, liquid nitrogen or electrothermia (figs. 7 and 8), to subsequently fill the cavity with autologous bone graft, restoring the anatomy of the region (fig. 9) and reestablishing the function (figs 10 and 11).
Fig. 7 – via de acesso à região póstero-medial da cabeça femoral.
Fig. 7 – access route to the posteromedial region of the femoral head.
Fig. 8 – curetagem intralesional, seguida de eletrotermia.
Fig. 8 – Intralesional curettage, followed by electrothermia.
Fig. 9 – enxerto ósseo autólogo já integrado. Fig. 10 – Função de flexão com carga dos quadris.
Fig. 9 – autologous bone graft already integrated. Fig. 10 – Load-bearing flexion function of the hips.
Fig. 11 Abdução do quadril, após um ano da cirurgia.
Fig. 11 Hip abduction, one year after surgery.
Chondroblastoma, despite being a benign lesion, in addition to local recurrence, can evolve into pulmonary metastases (figs. 12 and 13) which remain histologically benign, with the indication of additional chemotherapy being controversial, leaving only the excision of the metastases as treatment. as they do not respond to chemotherapy or radiotherapy.
Fig. 12 – radiografia do tórax, com múltiplos nódulos.
Fig. 12 – chest x-ray, with multiple nodules.
Fig. 13 – tomografia do tórax, com treze anos de evolução.
Fig. 13 – CT scan of the chest, with thirteen years of evolution.
In our experience we had two cases of osteoblastoma and one case of chondroblastoma with secondary lung disease. In this case of chondroblastoma, thoracotomy was performed and numerous pulmonary nodules were found, which persist to this day. This patient, at the time of diagnosis of metastases, presented with hypertrophic pulmonary osteopathy. He did not undergo any additional treatment and is asymptomatic to this day, thirteen years later (figs. 14 and ’15) and fifteen years after surgery (figs. 16 and 17).
Fig. 14 – Abdução apos 8 anos. Fig. 15 – Fexão com carga após 13 anos.
Fig. 14 – Abduction after 8 years. Fig. 15 – Loaded bending after 13 years.
Fig. 16 – Abdução após 15 anos. Fig. 17 Flexão com carga após quinze anos da cirurgia.
Fig. 16 – Abduction after 15 years. Fig. 17 Flexion with weight fifteen years after surgery.

Currently, the patient is well and asymptomatic, 24 years after surgery for the femoral injury and 22 years after the removal of some of the metastatic nodules.

The prognosis can be guarded for both local recurrence and orthopedic complications such as joint degeneration and growth deficit.

See the article published in 1995.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

Phone: +55 11 3231-4638  Cell:+55  11 99863-5577   Email:  drpprb@gmail.com

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Simple Bone Cyst

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Simple Bone Cyst

1. Definition

Unicameral cavity filled with clear or bloody fluid and limited by a membrane of variable thickness, with vascularized connective tissue showing osteoclastic giant cells and some areas with recent or old hemorrhage or fissures with cholesterol-rich content (OMS)

Simple Bone Cyst

2. Incidence

In our musculoskeletal tumor clinic, we observed a predominance of cases in the age group between 5 and 15 years, with a slight predominance of cases in males, and the majority involving the proximal metaphyseal region of the humerus and femur. The vast majority are referred due to an episode of fracture caused by trauma at the site of the injury or as an x-ray finding during an eventual x-ray taken due to some trauma suffered by the patient.

3. Etiology

Although its recognition from a radiographic point of view is simple, its etiology is still unknown. Our hypothesis is that this is a vascular phenomenon. In several cases, when they are treated with infiltration, we inject contrast and observe the existence of vascular fistulas associated with the persistence of the lesion, figures 1 to 3 and video 1.

Figura 1: Cisto ósseo unicameral. Figura 2: Infiltração com contraste, Figura 3: Preenchimento do cisto e de vasos que drenam a cavidade.
Figure 1: Unicameral bone cyst. Figure 2: Infiltration with contrast, Figure 3: Filling of the cyst and vessels that drain the cavity.

4. Clinical Assessment

Most patients present asymptomatically, and fractures are often the reason for their first consultation with an orthopedist. Some patients report sporadic episodes of pain or functional limitation before the presence of a bone cyst is diagnosed. Figure 4 illustrates its characteristics.

Figura 4: Diagrama do C.O.S.
Figure 4: COS Diagram

5. Radiographic Characteristics

The Simple Bone Cyst presents as a radio-transparent lesion in the metaphyseal region of long bones, centrally located, mainly in the proximal region of the humerus and femur and close to the epiphyseal line. They are well-defined lesions, with sclerotic edges, rarely crossing the limits of the cortex or the limits of the bone, expanding, thinning the cortex, but almost never breaking it. In some cases, the “fallen fragment” sign can be observed, which represents fragments of the cortical wall loose within the cyst.

6. Differential diagnosis

The main differential diagnoses are aneurysmal bone cyst, cortical fibrous defect / non-ossifying fibroma, eosinophilic granuloma, juxta-articular bone cyst, fibrous dysplasia, among others, figures 5 to 11. 

Figura 5: Cisto ósseo aneurismático
Figure 5: Aneurysmal bone cyst
Figura 6: Defeito fibroso cortical. Figura 7: Granuloma eosinófilo.
Figure 6: Cortical fibrous defect. Figure 7: Eosinophilic granuloma.
Figura 8: Cisto ósseo justa articular (ganglion). Figura 9: Após injeção de contraste.
Figure 8: Juxta-articular bone cyst (ganglion). Figure 9: After contrast injection.
Figura 10: Displasia fibrosa do colo femoral. Figura 11: Mancha café com leite da síndrome de Albright.
Figure 10: Fibrous dysplasia of the femoral neck. Figure 11: Café au lait spot from Albright syndrome.

7. Treatment

COS treatment depends on its location and size, in the vast majority of cases it can be conservative and non-operative. In general, treatment for the upper limb is less surgical and more conservative, whereas treatment for the lower limb tends to be more surgical, in an attempt to avoid a fracture. The classic treatment consists of infiltrations with corticosteroids (depomedrol), observing whether or not bone content is formed inside. If there is an imminent fracture in a load-bearing bone, we should seriously consider the possibility of intralesional treatment by filling the cavity with an autologous graft, preferably, figures 12 to 34.

Figura 12 à 17: Evolução natural de cisto ósseo simples da fíbula que não é osso de carga.
Figure 12 to 17: Natural evolution of a simple bone cyst of the fibula that is not a load-bearing bone.
Figura 18: lesão insuflativa da ulna. Figura 19: fratura do tornozelo. Figura 20: Cisto na pelve. Figura 21: Fratura do fêmur em criança. Figura 22: Fratura do colo femoral em adulto jovem.
Figure 18: Insufflation injury of the ulna. Figure 19: ankle fracture. Figure 20: Cyst in the pelvis. Figure 21: Femur fracture in a child. Figure 22: Femoral neck fracture in a young adult.
Figura 23: Dificuldade de fixação em criança em crescimento. Figura 24: Fratura de fêmur em adolescente.
Figure 23: Difficulty in fixation in a growing child. Figure 24: Femur fracture in a teenager.
Figura 25: Cisto unicameral no fêmur. Figura 26: Rx em perfil. Figura 27: infiltração. Figura 28: Contraste confirmando uma cavidade única. Figura 29: Segunda infiltração de C.O.S. do úmero, agora com septação.
Figure 25: Unicameral cyst in the femur. Figure 26: X-ray in profile. Figure 27: infiltration. Figure 28: Contrast confirming a single cavity. Figure 29: Second COS infiltration of the humerus, now with septation.
Figura 30 e 31: Grave fratura afundamento em cisto ósseo do fêmur.
Figure 30 and 31: Severe sinking fracture in femoral bone cyst.
Figura 32 e 33: Fixação com placa e enxerto autólogo. Figura 34: Boa função de flexão do joelho, com carga total.
Figure 32 and 33: Fixation with plate and autologous graft. Figure 34: Good knee flexion function, with full load.

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

Phone: +55 11 3231-4638  Cell:+55  11 99863-5577   Email:  drpprb@gmail.com

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Biopsy – Concept – Types

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Para melhor compreensão, sugerimos que leia primeiramente os capítulos: 

https://oncocirurgia.com.br/introducao-ao-estudo-dos-tumores-osseos/

https://oncocirurgia.com.br/diagnostico-dos-tumores/

Biopsy Considerations

Biopsy – Concept – Types – Indications – Planning

1.  Only after the clinical evaluation, with careful history taking and clinical examination, which will allow us to raise diagnostic hypotheses, should we request additional tests.

With the analysis of complementary exams, we should verify:

A- If our hypotheses are compatible with the tests and continue to qualify as possible diagnoses; 

B- A new hypothesis has appeared, which we had not thought of, and we will have to redo our clinical reasoning.

C- If the exams are correct, well done, images centered on the lesion, with good quality or we will have to repeat them.

          2.  Diagnosis hypotheses must first be made through clinical examination, laboratory tests and imaging.

    3.  Pathology   must be used as a  “tool”  to  confirm  or  not confirm the  suspected diagnosis.

If the anatomopathological examination reveals a diagnosis that was not on our list, we must reanalyze the case, redo our reasoning. If there is no  clinical, radiological and anatomopathological correlation  , something may be wrong and we will need to review it together, in a multidisciplinary team, to determine the best course of action. New biopsy?

4.  To reason about the diagnosis, it is first necessary to frame the condition we are analyzing within the five chapters of pathology, figures 1 and 2.

Biopsy – concept – types – indications – planning

Figura 1: Reunião Multidisciplinar - oncocirurgião, radiologista, patologista, oncologista, radioterapeuta, psicólogo, assistente social, fisioterapeuta, enfermagem e outros profissionais envolvidos no caso, ura.
Figure 1: Multidisciplinary Meeting - oncosurgeon, radiologist, pathologist, oncologist, radiotherapist, psychologist, social worker, physiotherapist, nursing and other professionals involved in the case, ura.
Figura 2: Em nossa biblioteca cerebral devemos pesquisar os cinco volumes da PATOLOGIA: 1- Malformações Congênitas, 2-Transtornos Circulatórios, 3- Processos Degenerativos, 4- Inflamações e 5- Neoplasias.
Figure 2: In our brain library we must research the five volumes of PATHOLOGY: 1- Congenital Malformations, 2- Circulatory Disorders, 3- Degenerative Processes, 4- Inflammations and 5- Neoplasms.

5.  If we conclude that our patient has a neoplasm, we need to carry out the reasoning exercise already described in the Introduction to the Study of Tumors and Tumor Diagnosis chapters (Links:  https://oncocirurgia.com.br/introducao-ao-estudo- dos-tumores-osseos/   and   https://oncocirurgia.com.br/diagnostico-dos-tumores/  ).

After these steps, we can think of the biopsy as a  “tool”  for the definitive diagnosis.

Before we address the topic  “biopsy”,  let’s analyze some cases.

Patient  A : figures 3 and 4.

Figura 3: Tomografia com lesão na parede abdominal. Abaulamento do músculo reto anterior e espessamento da musculatura lateral, assinalado em amarelo.
Figure 3: Tomography showing injury to the abdominal wall. Bulging of the anterior rectus muscle and thickening of the lateral muscles, marked in yellow.
Figura 4: Tomografia, ultrassom e aspecto clínico. Paciente ictérico, asmático, com grande equimose, internado havia dias para investigação.
Figure 4: Tomography, ultrasound and clinical appearance. Jaundiced, asthmatic patient, with severe bruising, hospitalized for days for investigation.

Thirty days ago, they requested a biopsy of an abdominal wall lesion on a patient admitted for investigation.

The patient’s doctor found me in the radiology room, analyzing the CT scan.

Following the  “how I think”  about injuries I asked myself: – what structures form the abdominal wall? The. skin  (squamous cell carcinoma, basal cell carcinoma, melanoma) ; B. subcutaneous  (lipoma, liposarcoma) ; w. muscular fascia  (desmoid fibroma) ; d. striated muscle  (fibroma, fibrosarcoma, desmoid fibroma, rhabdomyosarcoma) ; It is. vessel  (hemangioma, leiomyosarcoma) ; f. peritoneum and abdominal cavity  (no longer my jurisdiction).

It seemed like an extensive lesion and I suggested that I look for a surgeon in the area, as I wouldn’t know how to drive if it was a malignant neoplasm. Ideally, the biopsy should be performed by the person who will operate on the patient.

He told me that the patient was jaundiced, an ultrasound and several laboratory tests had been performed, insisting that I perform a biopsy. I asked him some information and as I didn’t know how to find out, I suggested that we visit the bed. We could extract the clinical history and examine the patient.

The patient reported being asthmatic and reported that the symptom began abruptly after a coughing fit eleven days ago, in a sudden change of weather, cold and drizzling. He had severe pain in the anterior wall of the abdomen, where a “ball” appeared. The bulging and pain were decreasing and the side wall had hardened.

Leaving the room, I suggested that we not do a biopsy, that we discharge the patient, that the jaundice with elevated bilirubin was the result of a large hematoma that had infiltrated the lateral wall, due to the spontaneous rupture of the anterior rectus abdominis. This lesion was already undergoing repair and the biopsy would only show the scarring inflammatory process  (with the risk of proliferative myositis).

Still not convinced, he asked me if I had ever seen a case of spontaneous rupture of the rectus abdominis muscle. I answered no, but that was what common sense said. Going down the stairs we met a general surgeon and I asked him about the matter. This clarified that it was common in patients with chronic bronchitis who were taking corticosteroids, as was the case with our patient.  The  clinical history  made the diagnosis.

Patients  B  and  C : Figures 5 and 6.
Pacientes B e C: Figuras 5 e 6.
Figura 5: Radiografia da pelve esquerda com lesões de rarefação no ramo ílio-isquiático, paciente mostrando a lesão, destacada em vermelho.
Figure 5: Radiograph of the left pelvis with rarefaction lesions in the ilio-ischial branch, patient showing the lesion, highlighted in red.
Figura 6: Lesão na coxa assinalada em azul, reação periosteal ao redor de corpo estranho, destacada em amarelo, ponta de lança de portão, circundada em vermelho.
Figure 6: Thigh injury marked in blue, periosteal reaction around a foreign body, highlighted in yellow, gate spearhead, circled in red.

Patients  B : Figure 5.

At the outpatient clinic, the resident asks:

– “By which access route should we perform the biopsy?”

I see the image and ask: – How old is the patient?

– “Um… Dona Maria, how old are you?”

 I reflect in silence, evaluating the learner’s lack of knowledge. The patient responds 67 years old DOCTOR!

… Sixty-seven years, multiple lesions, metastasis? Multiple myeloma? Brown tumor of hyperparathyroidism? – How long has she had symptoms?

– “Um… Dona Maria, how long have you had this problem?”

In the medical record I see symptoms of pain in the  ischial tuberosity noted , measurements of Ca ++ , P ++ , FA, Na + , K + , protein electrophoresis, blood count, ESR, blood glucose, urea, creatinine, ultrasound, x-rays,…, …

When examining the patient, I observed that the “tumor” is  anterior , in the inguinal region, and not  posterior , as noted in the medical record, “ischial tuberosity”.  The patient had not been examined !!! She had an inguinal-crural hernia. Pelvic x-ray images represent gas from the intestine. The “biopsy” would result in intestinal perforation.  The  physical examination  made the diagnosis. 

Patient  C : Figure 6.

Passing through the emergency room, the person on duty asks:

– “Doctor, what tumor do you think this patient has? Can we schedule the biopsy?”

The resident knew nothing about the history and had only taken the frontal x-ray!!! When asked, the patient reports that the inflammatory symptoms began six months ago, with hot pain and the release of purulent secretions. When it was open, secreting, the symptoms improved. When he closed the fistula it started to swell, hurt and he had a fever.

With difficulty, as the patient often withholds information, we learned that he had been injured in the thigh two years ago, when he jumped over the guardrail of a house, which bled a lot, but did not seek treatment  ( clinical history ) . We requested a lateral x-ray which confirmed that it was a foreign body. The spear tip of the grid was surrounded by solid periosteal reaction, giving the false impression of a sclerotic tumor.  Appropriate imaging confirmed   the diagnosis.

After these important considerations, we will study the controversial topic of biopsy.

After these important considerations, we will study the controversial topic of biopsy.

WE NEED:

1-  Define the hypotheses of possible diagnoses, for our case, firstly with the  clinical history  and  physical examination ;

2-  Carry out  laboratory and imaging tests, to  corroborate  or  not  our hypotheses,  our reasoning  and

3-  Only after these steps can we perform the biopsy, for the pathology to “ recognize the signature ” of the diagnosis, previously thought out with our anamnesis, physical, laboratory and imaging examination.

“Pathological anatomy is not a short path to diagnosis. We must always correlate it with the clinic, laboratory and imaging tests”.

Figura 7: O médico precisa sentir o paciente.
Figure 7: The doctor needs to feel the patient.
Figura 8: A clínica é a base, que tem o maior peso. Os dados de imagem em equilíbrio com a patologia equilibram a pirâmide, definindo o DIAGNÓSTICO preciso.
Figure 8: The clinic is the base, which has the greatest weight. Image data in balance with pathology balances the pyramid, defining the precise DIAGNOSIS.
Figura 9: Quatro itens devem ser considerados em relação à biópsia.
Figure 9: Four items must be considered in relation to biopsy.
Figura 10: A amostra deve ser representativa da lesão, em qualidade e quantidade.
Figure 10: The sample must be representative of the lesion, in quality and quantity.
Figura 11: A escolha de cada tipo deve ser feita com critério.
Figure 11: The choice of each type must be made carefully.
Figura 12: Lesões que podem permitir a ressecção-biópsia. É preciso analisar caso a caso. Uma equipe multidisciplinar é fundamental.
Figure 12: Lesions that may allow resection-biopsy. It needs to be analyzed case by case. A multidisciplinary team is essential.

Regarding biopsy, we can subdivide musculoskeletal lesions into three groups:   

  1. Cases in which CLINICAL – RADIOLOGICAL diagnosis  (image)  is sufficient for diagnosis and treatment, and biopsy is not indicated.
  2. Cases that may not require this procedure due to difficulty in histological diagnosis, and due to the characteristics of  clinical  and  radiological aggressiveness  , the necessary surgical procedure should not be altered.
  3. Cases that require pathological confirmation for chemotherapy treatment prior to surgery

We will discuss the three groups, analyzing some examples, figures below.

GROUPS 1 and 2 : Biopsy is not necessary or does not change management.

  1a . OSTEOMA, figures 13 to 18.

IDENTITY:  Benign, well-defined neoplastic lesion, characterized by a homogeneous, sclerotic and dense tumor, mature bone tissue. It’s bone within a bone.

Figura 13: Paciente com 43 anos de idade, apresentando tumor no crâneo havia oito anos, indolor, que dificultava para pentear o cabelo. Radiografia com lesão esclerótica homogenia.
Figure 13: 43-year-old patient, with a painless skull tumor for eight years that made it difficult to comb her hair. Radiograph with homogeneous sclerotic lesion.
Figura 14: Tomografia exibindo osteoma no crâneo.
Figure 14: Tomography showing osteoma in the skull.
Figura 15: Radiografia com osteoma na falange proximal do terceiro dedo.
Figure 15: Radiograph showing osteoma in the proximal phalanx of the third finger.
Figura 16: Radiografia com osteoma na cabeça femoral. Enostose assintomática, achado casual em radiografia do quadril.
Figure 16: Radiograph showing osteoma in the femoral head. Asymptomatic enostosis, casual finding on hip radiography.
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Figure 17: Osteoma in the vertebral body, asymptomatic. Casual finding, observation and follow-up.
Figura 18: Osteoma na região frontal do crâneo. Indicação cirúrgica por alteração estética.
Figure 18: Osteoma in the frontal region of the skull. Surgical indication due to aesthetic changes.

These lesions are well-defined, homogeneous, without symptoms. They are diagnosed by occasional imaging findings or by presenting aesthetic changes. Occasionally, they may be symptomatic, as in a case where the nasal cavity was obstructed, making breathing difficult. The diagnosis is clinical and radiological, and does not require a biopsy. Treatment is restricted to observation and monitoring. They are rare and occasionally operated on. 

See:   http://osteoma     and     http://osteoma of the skull

1b . OSTEOID OSTEOMA, figures 19 to 26.

IDENTITY:  Benign neoplastic lesion, characterized by a circumscribed tumor, up to approximately one centimeter in diameter, which presents a central osteoid niche, surrounded by a halo of sclerosis and located in the cortex of the long bones, the most compact part.

Figura 19: TC e Radiografias de osteoma osteóide da região trocanteriana do fêmur. Lesão lítica, com nicho central e halo de esclerose, assinalada em amarelo e cortical marcada com perfuração por fio de Kirchner, assinalada em vermelho.
Figure 19: CT and radiographs of osteoid osteoma of the trochanteric region of the femur. Lytic lesion, with central niche and halo of sclerosis, marked in yellow and cortical marked with Kirchner wire perforation, marked in red.
Figura 20: Radiografia de osteoma osteóide no colo femoral. Lesão lítica, com nicho central e halo de esclerose, seta vermelha.
Figure 20: Radiograph of osteoid osteoma in the femoral neck. Lytic lesion, with central niche and halo of sclerosis, red arrow.

The femoral neck region is covered by a thin periosteum that does not present a periosteal reaction. This makes it difficult to locate the lesion during surgery.

Making a hole in the cortical bone, close to the lesion, guided by radioscopy, will facilitate the operation.

After this marking, we perform a tomography to measure the distance from the hole to the center of the lesion, locating it. See the complete technique at:  http://osteoid osteoma resection technique   

Figura 21: Tomografia com osteoma osteóide na região medial do colo femoral. Neste caso a marcação coincidiu com o centro da lesão, o que facilita, mas não é o ideal, pois perfura a lesão.
Figure 21: Tomography showing osteoid osteoma in the medial region of the femoral neck. In this case, the marking coincided with the center of the lesion, which makes it easier, but is not ideal, as it pierces the lesion.
Figura 22: Imagens com osteoma osteóide da tíbia e do pedículo da coluna vertebral. Não há indicação de biópsia e sim de ressecção-biópsia.
Figure 22: Images with osteoid osteoma of the tibia and spinal pedicle. There is no indication for biopsy, but rather for resection-biopsy.
Figura 23: Osteoma osteóide da cortical anterior da tíbia. Não ha indicação de biópsia.
Figure 23: Osteoid osteoma of the anterior cortical bone of the tibia. There is no indication for biopsy.
Figura 24: Osteoma osteóide periosteal da cortical interna do rádio. Não há indicação de biópsia.
Figure 24: Periosteal osteoid osteoma of the inner cortex of the radius. There is no indication for biopsy.
Figura 25: Osteoma osteóide do calcâneo. Não há indicação de biópsia.
Figure 25: Osteoid osteoma of the calcaneus. There is no indication for biopsy.
Figura 26: Osteoma osteóide sub talar do calcâneo. Não há indicação de biópsia.
Figure 26: Subtalar osteoid osteoma of the calcaneus. There is no indication for biopsy.

Osteoid osteoma is a lesion of the cortical bone. In the spine, it occurs in the pedicle, which is the most compact, hardest part, resembling the cortex.

It has a central niche with a halo of sclerosis around it and does not exceed one centimeter.

There is no such thing as a “giant osteoid osteoma”, larger than 1.5 cm, as in this situation there is cortical erosion, there is no delimitation by the sclerosis halo and, although it may present similar histology, we are dealing with an osteoblastoma, which is a benign lesion. , but locally aggressive. Osteoblastoma may or may not be associated with an aneurysmal bone cyst and may also require a differential diagnosis with teleangiectatic osteosarcoma. Read also: http://osteoid osteoma 

1c . OSTEOCHONDROMA, figures 27 to 32.

IDENTITY:  It is an exostosis in which the central cancellous bone continues with the medullary of the affected bone and the dense peripheral, cortical layer of the tumor continues with the cortical layer of the affected bone. It presents with an enlarged,  sessile,  or narrow,  pedicled base . It can be single or multiple  (hereditary osteochondromatosis).

Osteochondromas require surgical treatment when they alter aesthetics or function, displacing and compressing vascular-nervous structures, limiting movement or generating angular deformities. It is the most common benign bone lesion.

They generally grow while the patient is in the growth phase. When an osteochondroma increases in size after completion of skeletal maturity, it may mean post-traumatic bursitis or malignancy to chondrosarcoma and should be treated as such, resecting with an oncological margin. 

Solitary osteochondroma has a 1% malignancy rate. Multiple osteochondromatosis can reach 10%.

Figura 27: Exemplo de osteocondroma pediculado do fêmur e de osteocondroma pediculado da tíbia. A cortical do osso continua-se com a cortical da lesão e a medular do osso também se continua com a medular da lesão. Não há indicação de biópsia.
Figure 27: Example of pedicled osteochondroma of the femur and pedicled osteochondroma of the tibia. The bone cortex continues with the lesion cortex and the bone medullary bone also continues with the lesion medullary. There is no indication for biopsy.
Figura 28: Osteocondroma séssil da tíbia. Não há indicação de biópsia. Indicação de ressecção por bloqueio da flexão do joelho.
Figure 28: Sessile osteochondroma of the tibia. There is no indication for biopsy. Indication of resection by blocking knee flexion.
Figura 29: Osteocondromatose múltipla hereditária. Lesões múltiplas em irmãos, setas brancas, amarelas e azuis. Deformidade angular e encurtamento do membro superior.
Figure 29: Hereditary multiple osteochondromatosis. Multiple lesions in siblings, white, yellow and blue arrows. Angular deformity and shortening of the upper limb.
Figura 30: Osteocondroma múltiplos nos fêmures e nas tíbias. Epifisiodese medial da tíbia esquerda visando corrigir a deformidade angular.
Figure 30: Multiple osteochondromas in the femurs and tibias. Medial epiphysiodesis of the left tibia aiming to correct the angular deformity.
Figura 31: Osteocondroma séssil do fêmur, deslocando os vasos femorais. A cortical do osso continua-se com a cortical da lesão e a medular do osso também se continua com a medular da exostose.
Figure 31: Sessile osteochondroma of the femur, displacing the femoral vessels. The bone cortex continues with the lesion cortex and the bone medullary also continues with the exostosis medullary.
Figura 32: Osteocondroma da fíbula comprimindo lentamente a tíbia, durante o crescimento, e ocasionando deformidade angular em valgo e antecurvatum. Exostose no pé dificultando o uso de calçado. Peças e histologia.
Figure 32: Osteochondroma of the fibula slowly compressing the tibia during growth and causing angular deformity in valgus and antecurvatum. Exostosis in the foot making it difficult to wear shoes. Parts and histology.

The diagnosis of osteochondroma is clinical and radiological and does not require a biopsy for treatment. 

Read:  http://osteochondroma

1d . CONDROMA, figures 33 to 50.

IDENTITY:  Benign, painless, cartilage-forming tumor with foci of calcification in the short bones of the hands and feet, diagnosed by chance or due to deformity or fracture. It can be solitary or multiple  (enchondromatosis, Maffucci syndrome, Ollier disease).

Figura 33: Condromas das falanges, achado casual. Histologia de condroma, cartilagem madura.
Figure 33: Chondromas of the phalanges, casual finding. Chondroma histology, mature cartilage.
Figura 34: Condroma da falange com DOR. Consolidado após fratura havia cinco meses, tratada com imobilização. OPERAR?
Figure 34: Chondroma of the phalanx with PAIN. Healed after fracture five months ago, treated with immobilization. OPERATE?
Figura 35: Ressonância de condroma da falange após fratura há cinco meses, com DOR!
Figure 35: MRI of chondroma of the phalanx after fracture five months ago, with PAIN!
Figura 36: Cortical com insuflação fina causando dor. Captação de gadolínio. OPERAR?
Figure 36: Cortical with fine inflation causing pain. Gadolinium uptake. OPERATE?
Figura 37: Cortical com insuflações finas causando dor e desconforto. Indicação de curetagem e enxerto autólogo S/N.
Figure 37: Cortical with fine insufflations causing pain and discomfort. Indication of curettage and autologous graft S/N.
Figura 38: Acesso dorso lateral, curetagem cuidadosa da lesão, cavidade sem lesão, material cartilaginoso curetado e enxerto autólogo do ilíaco, para preenchimento da cavidade.
Figure 38: Dorso-lateral access, careful curettage of the lesion, cavity without injury, cartilaginous material curetted and autologous iliac graft to fill the cavity.

In the fingers and toes, cartilaginous lesions generally behave benignly.

The eventual unwanted evolution to chondrosarcoma, resulting from curettage surgery in these locations, does not compromise the possibility of a cure, as complete resection of the finger,  which is the treatment of chondrosarcoma , would continue to be possible.

CONTROVERSY :   CHONDROMA OR CHONDROSSARCOMA GRADE I?

        Chondroma occasionally occurs in the metaphysis of long bones  (distal femur, humerus and proximal tibia)  and limb roots  (shoulder, pelvis) . In these cases, it can be confused with bone infarction or grade I chondrosarcoma.

In occasional findings, as  the anatomopathological diagnosis  between chondroma and grade I chondrosarcoma  is controversial , it is preferable not to perform a biopsy and monitor clinically and radiographically whether there is progress.

Grade I chondrosarcoma is slow to evolve, which allows monitoring, enabling observation for a safe diagnosis of its activity or not.

The exams are repeated at one, three and six months, and then annually. The tumor must be treated surgically as  chondrosarcoma  at any time if comparison between images reveals changes in the lesion.

If the injury remains unchanged, the best course of action is to continue monitoring. Some patients ask until when? The answer is: – Always. Reevaluation should continue regardless, whether the patient undergoes surgery or not.

Treating an asymptomatic lesion, a casual finding, without changing the image with minor surgery is “ overtreatment”,  which will also require follow-up or worse, if the anatomopathological examination reveals malignant histology.

Exemplifying this conduct, we will analyze the following case, followed for 14 years, figures 39 to 42.

Figura 39: Paciente com dor na interlinha do joelho após esporte. Ressonância para estudo da articulação mostra achado casual de lesão na metáfise distal do fêmur. CONDROMA / CONDROSSARCOMA GRAU I? QUAL A MELHOR CONDUTA?
Figure 39: Patient with pain in the knee joint after sports. MRI to study the joint shows a casual finding of a lesion in the distal metaphysis of the femur. CHONDROMA / CHONDROSSARCOMA GRADE I? WHAT IS THE BEST CONDUCT?
Figura 40: Radiografia com lesão provavelmente cartilaginosa, na metáfise distal do fêmur. Achado casual: CONDUTA = OBSERVAÇÃO.
Figure 40: Radiograph showing a probably cartilaginous lesion in the distal metaphysis of the femur. Casual finding: CONDUCT = OBSERVATION.
Figura 41: Radiografia de controle após doze anos, queixa de dor recente no joelho. Calcificação na interlinha medial, paciente joga Squash! A dor não tem nada a ver com a lesão em acompanhamento, que continua inalterada.
Figure 41: Control x-ray after twelve years, complaint of recent knee pain. Calcification in the medial interline, patient plays Squash! The pain has nothing to do with the ongoing injury, which remains unchanged.
Figura 42: Controle após 14 anos de acompanhamento, sem biópsia. Inalterado e assintomático, discreta "sensação de? ..., desconforto, quando muda o tempo".
Figure 42: Control after 14 years of follow-up, without biopsy. Unchanged and asymptomatic, discreet "sensation of?..., discomfort, when the weather changes".

CHONDROMA or  CHONDROSSARCOMA?  In these cases  common sense  must prevail, he warns us that  the paper accepts any writing.

We must base ourselves on the clinical behavior of the lesion. Was there a change or not? If we choose to perform a biopsy, we can only add whether or not it is a  “cartilaginous lesion” . We cannot change our behavior:  OBSERVE OR  OPERATE AS CHONDROSSARCOMA .  To be safe, if we choose to operate, we must treat it surgically as chondrosarcoma, which is our only “ tool” , as they do not respond to chemotherapy or radiotherapy.

Continuing, let us analyze figures 43 to 50.
Figura 43: Lesão cartilaginosa latente, acompanhada desde 2003, sem a realização de biópsia, comportamento de condroma. Imagem em 2016 sem alteração. Conduta = manter o acompanhamento, se houver alteração deve ser tratada como condrossarcoma.
Figure 43: Latent cartilaginous lesion, monitored since 2003, without biopsy, chondroma-like behavior. Image in 2016 without changes. Management = maintain monitoring, if there is a change it should be treated as chondrosarcoma.
Figura 44: Lesão cartilaginosa no ramo ílio-púbico direito. Acompanhada havia dois meses, houve piora da imagem, com ruptura da cortical! Foi operado como condrossarcoma, com ressecção segmentar da lesão, sem a realização de biópsia, pois independente do resultado, fosse este condroma ou condrossarcoma o tratamento deve ser cirúrgico, com ressecção ampla.
Figure 44: Cartilaginous lesion in the right iliopubic branch. Followed up for two months, the image worsened, with cortical rupture! It was operated on as chondrosarcoma, with segmental resection of the lesion, without performing a biopsy, because regardless of the result, whether it was chondroma or chondrosarcoma, the treatment must be surgical, with wide resection.
Figura 45: Lesão cartilaginosa, focos de calcificação e erosão da cortical, em cuja amostra de biópsia ¨diagnosticou¨ CONDROMA. Operado com prótese convencional, sem ressecção com margem. Em pouco tempo houve evolução do tumor, CONDROSSARCOMA, levando a uma necessidade de hemipelvectomia, cirurgia ablativa.
Figure 45: Cartilaginous lesion, foci of calcification and cortical erosion, in which the biopsy sample ¨diagnosed¨ CHONDROMA. Operated with conventional prosthesis, without resection with margin. In a short time, the tumor, CHONDROSSARCOMA, evolved, leading to the need for hemipelvectomy, ablative surgery.
Figura 46: Lesão cartilaginosa do fêmur com todas as características de lesão agressiva. Deve ser tratada como CONDROSSARCOMA.
Figure 46: Cartilaginous injury of the femur with all the characteristics of an aggressive injury. It should be treated as CHONDROSSARCOMA.
Figura 47: Radiografia de lesão no úmero, com todas as características de agressividade da lesão cartilaginosa: dor, erosão da cortical, alargamento do canal medular por atividade do tumor - o comportamento não é latente: é ativo e agressivo. Na ressonância podemos acompanhar o trajeto da biópsia, contaminado pela implantação da neoplasia.
Figure 47: X-ray of a lesion in the humerus, with all the aggressive characteristics of a cartilaginous lesion: pain, cortical erosion, widening of the spinal canal due to tumor activity - the behavior is not latent: it is active and aggressive. On MRI we can follow the biopsy path, contaminated by the implantation of the neoplasm.
Figura 48: Laudo de biópsia realizada, revela tratar-se de lesão cartilaginosa sem atipias, nesta amostra recomendando nova biópsia? Precisa? Que conduta o médico tomaria se em uma eventual nova biópsia, a qual continuaria sendo uma amostra, continuasse a impressão de condroma? Deve-se considerar o comportamento evolutivo da lesão e tratar como condrossarcoma.
Figure 48: Biopsy report performed, reveals that it is a cartilaginous lesion without atypia, in this sample recommending a new biopsy? It needs? What action would the doctor take if in a possible new biopsy, which would still be a sample, the impression of chondroma continued? The evolutionary behavior of the lesion must be considered and treated as chondrosarcoma.
Figura 49: Paciente tratada como condrossarcoma. Peça de paciente operada sem nova biópsia. Podemos verificar em destaque a implantação da neoplasia agressiva nos tecidos moles, através do trajeto da biópsia.
Figure 49: Patient treated for chondrosarcoma. Part of a patient operated on without a new biopsy. We can clearly see the implantation of the aggressive neoplasm in the soft tissues, through the biopsy path.
Figura 50: Ressecção e reconstrução com endoprótese, no membro dominante de paciente odontóloga, após quatorze anos.
Figure 50: Resection and reconstruction with endoprosthesis, in the dominant limb of a dental patient, after fourteen years.
The message we want to leave is:

¨The doctor  can perform the biopsy , as it is an academic procedure, which gives him more support as to whether it is a cartilaginous lesion. But  you should not operate with a curettage technique , such as chondroma, as latent chondromas of long bones, casual findings, do not require surgical treatment but rather observation. The biopsy hinders this observation because we will not know whether the pain and changes in the image, which may occur after the biopsy, would be due to the aggression of the biopsy or whether it is a chondrosarcoma manifesting itself. In conclusion, if the doctor chooses to intervene,  he must operate on chondrosarcoma . We also remember that surgery, performed using any technique, will not eliminate the need for observation and monitoring¨.

Read:  http://chondrosarcoma or chondroma?

 1 and .  CHONDROBLASTOMA, figures 51 to 54.

IDENTITY: Benign epiphyseal  neoplastic lesion   of the growing skeleton  (1st and  2nd decades  ),  characterized by bone rarefaction, erosion of the articular cartilage with inflation, cartilaginous cells  (chondroblasts), giant cells  and foci of calcification.

Figura 51: Condroblastoma, tumor epifisário ou apofisário dos ossos longos DO ESQUELETO EM CRESCIMENTO.
Figure 51: Chondroblastoma, epiphyseal or apophyseal tumor of the long bones OF THE GROWING SKELETON.
Figura 52: Lesão com matriz cartilaginosa, epifisária, em adolescente (esqueleto em crescimento), halo de esclerose, erosão da cartilagem articular e da cortical óssea, com focos de calcificação = CONDROBLASTOMA.
Figure 52: Lesion with cartilaginous matrix, epiphyseal, in an adolescent (growing skeleton), halo of sclerosis, erosion of the articular cartilage and cortical bone, with foci of calcification = CHONDROBLASTOMA.
Figura 53: Lesão com matriz cartilaginosa, epifisária, em criança (esqueleto em crescimento), halo de esclerose, com focos de calcificação = CONDROBLASTOMA.
Figure 53: Lesion with cartilaginous matrix, epiphyseal, in a child (growing skeleton), halo of sclerosis, with foci of calcification = CHONDROBLASTOMA.
Figura 54: Acesso póstero medial à cabeça femoral, para permitir o tratamento cirúrgico da lesão com curetagem, eletro termia e reconstrução com enxerto autólogo do ilíaco.
Figure 54: Posteromedial access to the femoral head, to allow surgical treatment of the injury with curettage, electrothermia and reconstruction with an autologous iliac graft.

Adjuvant curettage and electrothermal surgery for this neoplasm, in these locations and in small-sized lesions, is nothing more than an incisional biopsy, in which the macroscopic appearance of cartilage allows complete curettage of the tumor. The presence of the pathologist in the surgery is useful to corroborate and assist the surgeon. Read:  http://chondroblastoma

 1f . SIMPLE BONE CYST – COS, figures 55 to 58.

IDENTITY: Pseudoneoplastic lesion, unicameral, surrounded by a membrane, well delimited, filled with serous fluid, central metaphyseal  location  , which does not exceed its width and occurs in children and adolescents.  

Figura 55: Cisto ósseo simples do úmero. Lesão bem delimitada que não ultrapassa a largura da metáfise. Descoberta devido à dor por micro fratura. Com o crescimento distancia-se da linha epifisial. Cavidade única, revestida por membrana contendo líquido seroso.
Figure 55: Simple bone cyst of the humerus. Well-defined lesion that does not exceed the width of the metaphysis. Discovery due to pain due to micro fracture. With growth it distances itself from the epiphyseal line. Single cavity, lined with a membrane containing serous fluid.
Figura 56: Cisto ósseo simples na fíbula. Esta é a única localização que pode eventualmente ser mais largo do que a metáfise, devido à cortical fina poder insuflar-se. A fíbula não é osso de carga, podemos observar. Com o crescimento afasta-se da linha epifisial e mineraliza, evoluindo para cura.
Figure 56: Simple bone cyst in the fibula. This is the only location that may eventually be wider than the metaphysis, due to the thin cortex being able to inflate. The fibula is not a load-bearing bone, we can see. As it grows, it moves away from the epiphyseal line and mineralizes, progressing towards healing.
Figura 57: Cisto ósseo simples da tíbia. Dor por tração da tuberosidade tibial pelo ligamento patelar, devido ao afilamento da cortical.
Figure 57: Simple bone cyst of the tibia. Pain caused by traction of the tibial tuberosity by the patellar ligament, due to cortical thinning.
Figura 58: Cisto ósseo simples. Cavidade única, bem delimitada, com conteúdo líquido envolto por uma membrana, seta em vermelho (captação de contraste apenas na periferia).
Figure 58: Simple bone cyst. Single, well-defined cavity, with liquid content surrounded by a membrane, red arrow (contrast capture only at the periphery).

Read: http://simple bone cyst 

          1g . JUSTAARTICULAR BONE CYST – GANGLION, figures 59 to 62.

IDENTITY:  Pseudoneoplastic lesion,  epiphyseal in location , unicameral, surrounded by synovial membrane, well defined and filled with serous fluid, which communicates with the adjacent joint.

Figura 59: Lesão epifisária de rarefação óssea bem delimitada. Ressonância sagital revelando pertuito na cartilagem articular comunicando o líquido da articulação com o do conteúdo da cavidade. GANGLION (cisto ósseo justa articular).
Figure 59: Well-defined epiphyseal bone rarefaction lesion. Sagittal resonance revealing a hole in the articular cartilage communicating the joint fluid with the contents of the cavity. GANGLION (just articular bone cyst).
Figura 60: Lesão homogênea, com conteúdo líquido (baixo sinal em T1 e Alto sinal em T2). Em sagital T1 com contraste observamos captação apenas na periferia da lesão, destacando a membrana sinovial secretora do líquido seroso que preenche a cavidade.
Figure 60: Homogeneous lesion, with liquid content (low signal on T1 and high signal on T2). In sagittal T1 contrast, we observed uptake only at the periphery of the lesion, highlighting the synovial membrane that secretes the serous fluid that fills the cavity.
Figura 61: Lesão epifisária de rarefação óssea bem delimitada. Ressonância coronal e axial destacando o aspecto homogêneo e circunscrito da lesão. Ganglion? Provavelmente não, pois não há comunicação com a articulação. Provável cárie óssea, sequela de processo inflamatório.
Figure 61: Well-defined epiphyseal bone rarefaction lesion. Coronal and axial resonance highlighting the homogeneous and circumscribed appearance of the lesion. Ganglion? Probably not, as there is no communication with the joint. Probable bone caries, sequelae of an inflammatory process.
Figura 62: Ressonâncias sagitais T1 e com contraste evidenciando a delimitação periférica da lesão, que não se comunica com a articulação. Lesão de conteúdo líquido homogêneo e muito pequena, pode ser tratada sem biópsia.
Figure 62: Sagittal T1 and contrast-enhanced MRI scans showing the peripheral delimitation of the lesion, which does not communicate with the joint. Lesion with homogeneous liquid content and very small, can be treated without biopsy.

These lesions do not require a biopsy for treatment.

          1h . CORTICAL FIBROUS DEFECT / NON-OSSIFYING FIBROMA, figures 63 and 64.

IDENTITY:  Pseudoneoplastic lesion in the  cortical bone  with precise limits, asymptomatic. Occasional find.

Figura 63: Radiografia com lesão circunscrita na cortical do fêmur. Na tomografia observamos que é homogênea, pequena, menor que 1.5 cm, delimitada por halo de esclerose. Defeito fibroso cortical operado por desconforto leve, devido à inserção do músculo adutor.
Figure 63: Radiograph with circumscribed lesion in the cortical bone of the femur. On the tomography we observed that it is homogeneous, small, less than 1.5 cm, delimited by a halo of sclerosis. Cortical fibrous defect operated for mild discomfort, due to the insertion of the adductor muscle.
Figura 64: Lesão circunscrita na cortical lateral da tíbia, maior que 1.5 cm. Neste fibroma não ossificante, observamos que a lesão se distancia da linha epifisial e ocorre discreta mineralização. Achado de exame, acompanhamento sem biópsia.
Figure 64: Circumscribed lesion in the lateral cortex of the tibia, greater than 1.5 cm. In this non-ossifying fibroma, we observed that the lesion distances itself from the epiphyseal line and slight mineralization occurs. Examination finding, follow-up without biopsy.
These lesions occur in the  cortical bone  and do not require a biopsy for treatment/monitoring.

1i . FIBROUS DYSPLASIA OF THE  TIBIA  / OSTEOFIBRODYSPLASIA, figures 65 to 70.

IDENTITY:  Pseudoneoplastic lesion in the  tibial diaphysis  with bone rarefaction of intermediate density, as if the bone had been  “erased” , with a ground-glass appearance. It can occur in more than one location. Its evolution is variable and can cause deformity, dedifferentiation or harmonious growth, stabilizing at skeletal maturity. 

Figura 65: Lesão diafisária em criança com um ano de idade, com aumento acentuado e deformidade progressiva em dezoito meses. OSTEOFIBRODISPLASIA.
Figure 65: Diaphyseal injury in a one-year-old child, with marked increase and progressive deformity in eighteen months. OSTEOFIBRODYSPLASIA.
Figura 66: Paciente operada sem biópsia prévia, com ressecção da lesão, controlando sua progressão e corrigindo a deformidade. Reconstrução biológica com enxerto autólogo e homólogo.
Figure 66: Patient operated on without prior biopsy, with resection of the lesion, controlling its progression and correcting the deformity. Biological reconstruction with autologous and homologous graft.
Figura 67: Paciente com cinco anos de idade. Em 1990, foi encaminhado para “amputação” devido a lesão na tíbia! Displasia fibrosa? Osteofibrodisplasia? Adamantinoma da tíbia? Conduta: OBSERVAÇÃO.
Figure 67: Five-year-old patient. In 1990, he was sent for “amputation” due to an injury to his tibia! Fibrous dysplasia? Osteofibrodysplasia? Adamantinoma of the tibia? Conduct: OBSERVATION.
Figura 68: Acompanhamento anual. Crescimento proporcional da lesão e alinhamento harmônico da perna. Conduta: OBSERVAÇÃO, sem biópsia.
Figure 68: Annual monitoring. Proportional growth of the lesion and harmonious alignment of the leg. Management: OBSERVATION, without biopsy.
Figura 69: Cintilografia e radiografias de 2016, após vinte e cinco anos de observação, sem biópsia. Paciente adulto, tíbia alinhada.
Figure 69: Scintigraphy and radiographs from 2016, after twenty-five years of observation, without biopsy. Adult patient, tibia aligned.
Figura 70: Aspecto clínico e funcional do paciente, após vinte e cinco anos de observação, sem biópsia. Não se deve tratar um rótulo.
Figure 70: Clinical and functional appearance of the patient, after twenty-five years of observation, without biopsy. A label should not be treated.

 1J . MYOSITIS OSSIFICANS, figures 71 and 72.

IDENTITY:  Injury located close to a bone and in soft tissues, related to previous trauma, whose ossification begins in the periphery. 

Figura 71: Dor na face medial da coxa esquerda havia oito meses, após trauma. Hipotrofia do quadríceps denotando lesão cônica. Radiografia e cintilografia revelando ossificação.
Figure 71: Pain on the medial side of the left thigh for eight months, after trauma. Quadriceps hypotrophy denoting conical injury. Radiography and scintigraphy revealing ossification.
Figura 72: Tomografia e ressonância evidenciando ossificação em tecidos moles, principalmente na periferia da lesão. A biópsia pode dar falso diagnóstico de osteossarcoma!
Figure 72: Tomography and resonance showing ossification in soft tissues, mainly on the periphery of the lesion. Biopsy can give a false diagnosis of osteosarcoma!

1k . SOFT TISSUE TUMOR –  SOME , figures 73 to 78.

IDENTITY:  Delimited, homogeneous lesions, with typical images, without contrast uptake or with uptake only in the periphery, can be operated on without prior biopsy, when the surgical approach would not be different, even in the case of a malignant neoplasm.

Figura 73: Tumor de tecidos moles da região glútea, homogêneo, densidade de gordura em T1, que continua com o mesmo aspecto na saturação. LIPOMA.
Figure 73: Soft tissue tumor of the gluteal region, homogeneous, fat density on T1, which continues with the same appearance at saturation. LIPOMA.
Figura 74: Ressonância confirmando ser tecido gorduroso homogêneo, captação de contraste apenas na periferia do tumor. Ressecção da lesão envolta em sua pseudocápsula. LIPOMA.
Figure 74: MRI confirming that it is homogeneous fatty tissue, contrast uptake only at the periphery of the tumor. Resection of the lesion enclosed in its pseudocapsule. LIPOMA.

Malignant soft tissue tumors would have the same surgical resection procedure, with the narrow margins presented in the case above and would be complemented with local radiotherapy.  Soft tissue sarcomas, to date, do not respond to chemotherapy nor show an improvement in the patient’s survival rate.

See:  http://soft tissue sarcomas / chemotherapy

Figura 75: Ressonância evidenciando lesão em tecidos moles no trajeto do nervo interósseo posterior. Sinal de tinel positivo. SCHWANNOMA. Ressecção biópsia, abrindo o perineuro e enucleando o tumor, que se desprende facilmente ("como o caroço do abacate").
Figure 75: MRI showing injury to soft tissues in the path of the posterior interosseous nerve. Positive tinel sign. SCHWANNOMA. Biopsy resection, opening the perineurium and enucleating the tumor, which detaches easily ("like an avocado pit").
Figura 76: Radiografia com tumor no cavo poplíteo. Ressonância em T1 e T2 desenhando a “cauda de cometa” em trajeto nervoso. Exérese da lesão que parece uma cebola. Abre-se cuidadosamente a bainha (“casca da cebola”) e a lesão é retirada por completo, sem lesar o nervo.
Figure 76: Radiograph showing tumor in the popliteal cavity. MRI in T1 and T2 drawing the “comet tail” in the nervous path. Excision of the lesion that looks like an onion. The sheath (“onion skin”) is carefully opened and the lesion is removed completely, without damaging the nerve.

A possible biopsy could cause nerve damage and would not change the management.

Figura 77: Tumor heterogêneo da região posterior da coxa, deslocando os vasos femorais e o nervo ciático. SARCOMA DE TECIDOS MOLES.
Figure 77: Heterogeneous tumor of the posterior region of the thigh, displacing the femoral vessels and the sciatic nerve. SOFT TISSUE SARCOMA.
Figura 78: Ressecção com margens exíguas, apenas com sua pseudocápsula, liberando-se o nervo da lesão. Patologia confirma a hipótese de lipossarcoma mixóide. Após a completa cicatrização da ferida operatória, é realizada a radioterapia adjuvante.
Figure 78: Resection with tight margins, with only its pseudocapsule, freeing the nerve from the lesion. Pathology confirms the hypothesis of myxoid liposarcoma. After complete healing of the surgical wound, adjuvant radiotherapy is performed.

Biopsy can be performed, it is academic, it complements the case studies, but surgical resection must prevail, even in the case of malignant neoplasia. Soft tissue sarcomas, to date, do not benefit from neoadjuvant treatment and ablative surgery does not alter survival.

GROUPS  3 : Biopsy is necessary for treatment  (surgery; with/without neoadjuvance) 

We need to emphasize that the biopsy must be  performed/ monitored  by the surgeon who will perform the surgery. Your presence is essential for this to be carried out in accordance with the surgery planning.

Transverse incisions should not be made, nor extensive incisions where there is no musculature for subsequent coverage, such as on the leg, for example. The suture should not have points far from the incision, as this will require a larger resection of tissue and much less more than one incision, figures 79 (tables A, B, C and D) and 80.

Figura 79: Quadro A - incisão transversa INADEQUADA; quadro B - incisão grande e larga na tíbia lesando a pata de ganso; quadro C - pontos de sutura distantes da linha da incisão e quadro D - uma, duas, TRÊS INCISÕES !!!
Figure 79: Chart A - INADEQUATE transverse incision; table B - large and wide incision on the tibia damaging the pes ansus; frame C - suture points distant from the incision line and frame D - one, two, THREE INCISIONS!!!
Figura 80: Duas incisões !!! Distantes e com nódulo subcutâneo de implantação de tumor de células gigantes !!!
Figure 80: Two incisions!!! Distant and with a subcutaneous nodule of giant cell tumor implantation!!!
 See the complete case of figure 80 at:  http://tgc-prótese intraepifisária
Figura 81: Duas incisões!!! Trajetos inadequados dificultando a ressecção com margem do condrossarcoma. Foi necessária uma ressecção extra articular e reconstrução com uma artrodese empregando-se uma prótese rígida de joelho, feita sob medida.
Figure 81: Two incisions!!! Inadequate routes making resection with chondrosarcoma margin difficult. An extra-articular resection and reconstruction with arthrodesis using a custom-made rigid knee prosthesis were necessary.
Figura 82: Incisão transversa!!! Trajeto inadequado dificultando a ressecção com margem deste condrossarcoma da pelve. Foi necessária uma ampla ressecção de pele nesta hemipelvectomia interna.
Figure 82: Transverse incision!!! Inadequate path making it difficult to resect this chondrosarcoma of the pelvis with margin. A wide skin resection was required in this internal hemipelvectomy.
See the complete case in figure 82 at: http://internal pelvectomy

Below, we exemplify two cases of biopsies performed correctly, figures 83 to 86.

Figura 83: BIÓPSIA CORRETA. Puntiforme, com agulha de Jamshid, permitindo a ressecção do tumor com margem, juntamente com o trajeto da biópsia.
Figure 83: CORRECT BIOPSY. Punctate, with a Jamshid needle, allowing resection of the tumor with a margin, along with the biopsy path.
Figura 84: Peça ressecada com margem, incluindo o trajeto da biópsia. Reconstrução com dispositivo de fixação interna extensível e autotransplante com a cartilagem de crescimento da fíbula.
Figure 84: Resected piece with margin, including the biopsy path. Reconstruction with an extensible internal fixation device and autotransplantation with fibula growth cartilage.
*See the complete case of figures 83 and 84 at:  http://growth cartilage transplant
Figura 85: Radiografia e ressonância de osteossarcoma. A seta indica o ponto correto para a coleta da biópsia.
Figure 85: Radiography and resonance of osteosarcoma. The arrow indicates the correct point for biopsy collection.
Figura 86: Cicatriz puntiforme de BIÓPSIA CORRETA, realizada com agulha de Jamshid, permitindo a ressecção do tumor com margem, juntamente com o trajeto da biópsia.
Figure 86: Punctate scar from CORRECT BIOPSY, performed with a Jamshid needle, allowing resection of the tumor with a margin, along with the biopsy path.
*See the complete case of figures 85 and 86 at:  http://partial rotational prosthesis
PLANNING AND EXECUTION OF BIOPSIES :  CONSIDERATIONS – HOW TO PERFORM 
Case 1 Considerations :  We will describe how we proceeded in this female patient, 40 years old, with pain in the right posterior superior iliac crest for six months, figures 87 to 116.
Figura 87: Radiografia de bacia obturatriz com lesão na crista ilíaca direita, seta e círculo em vermelho.
Figure 87: X-ray of the obturator pelvis with injury to the right iliac crest, arrow and circle in red.
Figura 88: Radiografia de bacia em alar com áreas de rarefação e outras de condensação (focos de calcificação?).
Figure 88: X-ray of the alar pelvis with areas of rarefaction and others of condensation (calcification foci?).
Figura 89: Tomografia axial evidenciando a lesão lítica agressiva no ilíaco direito, com erosão da cortical e tumor extra cortical com focos salpicados de condensação óssea.
Figure 89: Axial tomography showing the aggressive lytic lesion in the right iliac, with cortical erosion and extra-cortical tumor with speckled foci of bone condensation.
Figura 90: Tomografia coronal. Observamos a lesão na crista ilíaca, círculo vermelho e a região póstero inferior sem lesão, podendo-se preservar uma ponte sacro ilíaca, seta amarela.
Figure 90: Coronal tomography. We observed the lesion on the iliac crest, red circle and the inferior posteroregion without injury, with a sacroiliac bridge being able to be preserved, yellow arrow.
In the MRI analysis, we studied the involvement of the lesion, planned the surgical access and resection tactics with margin, and then chose the most appropriate and safe route for our biopsy, figures 91 and 92.
Figura 91: Ressonância axial exibindo os limites do tumor. A lesão extraóssea está delimitada internamente pelo peritônio, seta vermelha, externamente pelo músculo glúteo, seta amarela, recoberta pelo plano gorduroso, seta branca. O trajeto ideal para a biópsia deve ser pela crista ilíaca, seta azul.
Figure 91: Axial resonance showing the limits of the tumor. The extraosseous lesion is delimited internally by the peritoneum, red arrow, externally by the gluteal muscle, yellow arrow, covered by the fatty plane, white arrow. The ideal route for the biopsy should be through the iliac crest, blue arrow.
Figura 92: Ressonância coronal destacando a lesão, círculo vermelho e o plano de corte planejado, seta amarela.
Figure 92: Coronal resonance highlighting the lesion, red circle and the planned cutting plane, yellow arrow.
Thus, the planned resection is to be accessed through an incision following the iliac crest, dissecting externally through the fat plane and internally detaching the peritoneum. We intended to place the patient in the supine position, but while dressing the patient was anesthetized and placed in the prone position, which made the procedure difficult, in our opinion, figures 93 and 94.
Figura 93: Paciente anestesiada em decúbito prono, realizada a marcação da incisão por sobre a crista ilíaca, que permitisse abordar ambos os lados da lesão, linha azul.
Figure 93: Patient anesthetized in prone position, marking the incision above the iliac crest, which allowed both sides of the lesion to be approached, blue line.
Figura 94: Controle tomográfico da lesão, com o paciente em decúbito prono.
Figure 94: Tomographic control of the lesion, with the patient in the prone position.

The Rx operator argued that that position was the best and that we could easily obtain the material for the histological study and… made an X where he would obtain the sample! Figures 95 and 96.

Figura 95: Marca onde pretendiam puncionar! Fora do trajeto planejado!
Figure 95: Mark where they intended to puncture! Off the planned route!
Figura 96: Tomo da pretendida punção! Como resgatar este trajeto na ressecção? Marca onde pretendiam puncionar! Fora do trajeto planejado!
Figure 96: Tome of the intended puncture! How can this path be recovered during resection? Mark where they intended to puncture! Off the planned route!
I explained that we should not change the direction of the planned surgical incision, as this would make internal access to the pelvis difficult. We advise you to puncture at the lateral point of the crest, despite the difficulty in angulating the needle, due to the prone position. This procedure is described as  ¨freezing biopsy¨ , figures 97 to 102.
Figura 97: Orientação para lateralizar o ponto de punção da biópsia, seta amarela.
Figure 97: Orientation to lateralize the biopsy puncture point, yellow arrow.
Figura 98: Controle tomográfico do novo posicionamento, o mais lateral possível, sobre a linha de incisão planejada.
Figure 98: Tomographic control of the new positioning, as lateral as possible, on the planned incision line.
Figura 99: Biópsia sob sedação e controle de tomografia.
Figure 99: Biopsy under sedation and tomography control.
Figura 100: Tomografia com esquema detalhando o planejamento da ressecção, com margem oncológica, incluindo o trajeto da biópsia.
Figure 100: Tomography with a diagram detailing the resection planning, with oncological margin, including the biopsy path.
Figura 101: Agulha de Tru-cut e material de anestesia local.
Figure 101: Tru-cut needle and local anesthesia material.
Figura 102: Estudo da amostra colhida pelo patologista, na sala de radiologia, conhecida como biópsia de congelação.
Figure 102: Study of the sample collected by the pathologist, in the radiology room, known as frozen section biopsy.
With the confirmation of a cartilaginous tumor, likely chondrosarcoma GII, we performed partial resection of the right pelvis, as planned, without neoadjuvance, figures 103 to 116.
Figura 102: Estudo da amostra colhida pelo patologista, na sala de radiologia, conhecida como biópsia de congelação.
Figure 103: Frozen biopsy report and subsequent paraffin review: Chondrosarcoma GII.
Figura 104: Paciente posicionada em decúbito lateral, marcação da incisão planejada na pele, com ressecção do trajeto da biópsia.
Figure 104: Patient positioned in lateral decubitus, marking of the planned skin incision, with resection of the biopsy path.
Figura 105: Incisão e hemostasia cuidadosa. Trajeto de biópsia, seta em azul.
Figure 105: Incision and careful hemostasis. Biopsy path, blue arrow.
Figura 106: Dissecção pelo plano gorduroso, que reveste os músculos glúteos a serem ressecados como margem. Hemostasia cuidadosa, não há sangramento.
Figure 106: Dissection through the fatty plane, which covers the gluteal muscles to be resected as a margin. Careful hemostasis, there is no bleeding.
Figura 107: Peça ressecada, face externa, plano gorduroso cobrindo a musculatura glútea ressecada.
Figure 107: Dried piece, external face, fatty plane covering the dry gluteal muscles.
Figura 108: Peça ressecada, face interna, margem exígua da pseudo cápsula, peritônio rebatido.
Figure 108: Resected piece, internal surface, narrow margin of the pseudo capsule, folded peritoneum.
Video 1: Exposure of the internal surface of the pelvis and delicate osteotomy, performed with minimally invasive drills.
Figura 109: Corte da peça ressecada, observando-se as margens marcadas com tinta nanquim.
Figure 109: Section of the dried piece, observing the margins marked with Indian ink.
Figura 110: Hematoxilina e eosina, histologia de Condrossarcoma grau II.
Figure 110: Hematoxylin and eosin, grade II chondrosarcoma histology.
Figura 111: Erosão óssea por neoplasia cartilaginosa.
Figure 111: Bone erosion due to cartilaginous neoplasia.
Figura 112: Neoplasia cartilaginosa, com mitoses atípicas e hipercromasia.
Figure 112: Cartilaginous neoplasm, with atypical mitoses and hyperchromasia.
Figura 113: Neoplasia cartilaginosa, com polimorfismo celular.
Figure 113: Cartilaginous neoplasm, with cellular polymorphism.
Figura 114: Laudo da peça cirúrgica. Condrossarcoma GII, com focos entre 5 a 10 % de GIII.
Figure 114: Report of the surgical specimen. Chondrosarcoma GII, with foci between 5 and 10% of GIII.
Case 2 Considerations :  Let’s now discuss the biopsy in this eleven-year-old patient, with pain and a tumor in the left thigh for two weeks. Probable osteosarcoma, figures 115 to 118.
Figura 115: Radiografia de frente com lesão na face lateral da metáfise distal do fêmur esquerdo, seta em vermelho e face posterior, radiografia em perfil.
Figure 115: Frontal radiograph with lesion on the lateral aspect of the distal metaphysis of the left femur, arrow in red and posterior aspect, lateral x-ray.
Figura 116: Tomografia axial destacando a lesão que ocupa a região central do osso e ultrapassa a cortical nas faces anterior, lateral e posterior, lesão osteoblástica, agressiva.
Figure 116: Axial tomography highlighting the lesion that occupies the central region of the bone and goes beyond the cortex on the anterior, lateral and posterior surfaces, an aggressive, osteoblastic lesion.
Figura 117: Ressonância coronal T1, com supressão de gordura destacando a extensão medular da lesão, que compromete a cartilagem de crescimento.
Figure 117: Coronal T1 resonance, with fat suppression highlighting the medullary extension of the lesion, which compromises the growth cartilage.
Figura 118: Ressonância axial T1, com supressão de gordura, lesão intramedular e extra cortical. Seta amarela aponta a fáscia lata. A seta amarela indica o trajeto adequado para a biópsia.
Figure 118: Axial T1 resonance, with fat suppression, intramedullary and extra-cortical lesion. Yellow arrow points to the fasciae latae. The yellow arrow indicates the appropriate path for the biopsy.

We very frequently see patients with biopsy scars performed in the anterolateral region of the distal metaphysis of the femur. The  red arrow  points to the fascia lata, which is most often interrupted by the biopsy path, carried out by professionals who will not operate on the patient, making it difficult to cover future surgery and the function of this limb that will need to be reconstructed.

The  yellow arrow  indicates the posterolateral path, most suitable for biopsy and reconstruction, providing the best coverage and function.

To perform the biopsy using this route, the appropriate positioning of the patient is in the prone position, figures 119 to 122.

Figura 119: Paciente em decúbito prono, para facilitar a realização da biópsia, posteriormente à inserção da fáscia lata, seta em vermelho. A Seta amarela destaca o controle tomográfico da posição.
Figure 119: Patient in prone position, to facilitate the biopsy, after insertion of the fascia lata, red arrow. The yellow arrow highlights the tomographic control of the position.
Figura 120: Paciente sob sedação, anestesia local e controle tomográfico do procedimento.
Figure 120: Patient under sedation, local anesthesia and tomographic control of the procedure.
Figura 121: Biópsia realizada abaixo da fáscia lata. Setas em vermelho, controle tomográfico do procedimento, seta amarela.
Figure 121: Biopsy performed below the fascia lata. Red arrows, tomographic control of the procedure, yellow arrow.
Figura 122: Patologia de congelação realizada atesta neoplasia maligna de grandes células, provável osteossarcoma.
Figure 122: Frozen section pathology performed attests to a large cell malignancy, likely osteosarcoma.

For the treatment of tumors of the distal end of the femur, such as this lesion, with this degree of involvement and location, we recommend biopsy as described and neoadjuvant induction chemotherapy, resection with oncological margin and reconstruction with modular prosthesis and adjuvant chemotherapy.

The patient in this example is out of treatment, with excellent function, and the complete case can be seen at Link:  http://osteosarcoma-length discrepancy  .

The performance of musculoskeletal biopsy, aiming at the diagnosis and adequate treatment of neoplasms, must be very well planned and carried out by experienced professionals.

“Carrying out musculoskeletal biopsies, aiming at the diagnosis and adequate treatment of neoplasms, must be very well planned and carried out by experienced professionals and with the participation of the surgeon who will be managing the case”. 

Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

Phone: +55 11 3231-4638  Cell:+55  11 99863-5577   Email:  drpprb@gmail.com

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Extendable internal fixation device

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Extendable internal fixation device. The authors present a dynamic internal fixation method that consists of a stainless steel piece that has a flap adapted to the bone that will be stabilized and a channel that will contain one of the ends of the plate to be used in osteosynthesis. This fixation prevents rotational deviations, valgus, varus, antecurvatum and retrocurvatum, but does not block bone growth of the epiphysis that is fixed. This device, called “device for extensible internal fixation”, was developed at the “Pavilhão Fernandinho Simonsen”, by the Oncological Orthopedics Group of the Department of Orthopedics and Traumatology of Santa Casa de Misericórdia de São Paulo (DOT-SCMSP-SP) and is indicated for the surgical treatment of selected cases of aggressive tumor lesions, and can also be used in the treatment of other conditions, such as congenital malformations and sequelae of trauma or infections that require reconstructions with dynamic stabilization, without blocking bone growth.

Extendable internal fixation device

INTRODUCTION

The search for biological solutions that make it possible to resolve bone defects, caused by locally aggressive tumor or pseudotumor lesions, congenital malformations, traumas and infections, has increasingly aroused the interest of orthopedists(1).

Advances in therapeutic resources in the treatment of malignant bone tumors have provided greater survival and even the prospect of a cure for patients. On the other hand, there is the inherent complication of endoprostheses over time. The correlation of these factors therefore requires the improvement of biological reconstruction methods that aim to be definitive(1-4). Reviewing the literature regarding bone reconstructions in the developing skeleton, it can be seen that this subject is of current interest(1,2,4-6).

Fig. 1 – Arteriografia do fêmur, mensuração de 20cm de ressecção. Arteriografia da fíbula e esquema do enxerto vascularizado.
Fig. 1 – Arteriography of the femur, measurement of 20cm of resection. Arteriography of the fibula and diagram of the vascularized graft.
However, we did not find any publication that made reference to any form of internal fixation of long bones that would allow stabilizing the epiphysis with the diaphyseal segment (fixing the epiphyseal plate), but at the same time would not block its growth. Such a mechanism should somehow allow the osteosynthesis system to slide, so as not to impede bone growth in the stabilized segment. As a result of treating a patient with Ewing’s sarcoma, we developed an internal fixation device that maintains the stabilization of the reconstruction and, at the same time, allows the bone to grow, either through its own epiphyseal plate or through the epiphyseal plate of the bone graft. transported or transplanted by microsurgical technique. This device prevents rotational deviations, valgus, varus, antecurvatum and retrocurvatum, but does not block bone growth of the epiphyseal plate that has been stabilized. For better understanding, we will present a description of the first case treated with this device, as well as exemplifying its use and possibility of adaptation to other bone segments.
Fig. 2 – Acesso medial, facilitando as anastomoses. Detalhes da peça ressecada, da placa angulada e das anastomoses.
Fig. 2 – Medial access, facilitating anastomoses. Details of the resected piece, the angled plate and the anastomoses.
Fig. 3 – Após 8 meses da 1ª cirurgia – peças de aço inoxidável – encaixe da placa permitindo o deslizamento – RX após a 2ª cirurgia.
Fig. 3 – 8 months after the 1st surgery – stainless steel parts – plate fitting allowing sliding – RX after the 2nd surgery.

DESCRIPTION OF THE TECHNIQUE

In February 1999, the nine-year-old male LCAA patient was undergoing preoperative chemotherapy treatment for diaphyseal Ewing sarcoma of the right femur at the Pediatric Hematology and Oncology Service of the Department of Pediatrics and Child Care of Santa Catarina. Casa Misericórida de São Paulo.

Fig. 4 – Acesso medial para retirada dos parafusos da placa angulada. Colocação da lâmina curva entre o fêmur e a placa. Aposição da segunda lâmina por sobre a primeira e a placa. Fixação do dispositivo com parafusos de anterior para posterior.
Fig. 4 – Medial access to remove the angled plate screws. Placement of the curved blade between the femur and the plate. Place the second blade over the first and the plate. Fixing the device with screws from anterior to posterior.
With the favorable response to neo-adjuvant chemotherapy, we decided to resect the affected segment and perform a biological reconstruction solution with transplantation of a vascularized contralateral fibula, using a microsurgical technique. To assess the extent of spinal involvement, we performed radiographs, bone scintigraphy, tomography and magnetic resonance imaging, pre- and post-chemotherapy, which safely showed us the possibility of resection of the diaphyseal segment, with preservation of the growth plate of the affected femur. In surgical planning, we calculated 20cm of resection, performed arteriography, chose the vessels for the anastomoses (fig. 1) and opted for a medial approach to facilitate resection, the placement of an angled plate fixing the femoral epiphysis and diaphysis and the vascular anastomoses through microsurgery (fig. 2). Eight months later, the control radiograph showed the bone transplant was fully integrated, both in the proximal and distal segments (fig. 3). However, the fibula had not yet obtained the necessary thickening to dispense with the protection of the angled plate. This osteosynthesis with an angled plate, fixed to the diaphysis and distal epiphysis of the femur, acted to block the growth of the bone provided by the distal epiphyseal plate of the femur, in addition to preventing the transported fibula from receiving the appropriate load request, in order to react and thicken it. faster.
Fig. 5 – L.C.C.A., masculino, 9 anos. Dispositivo estabilizando a osteossíntese. Início do deslizamento da placa (aparece o primeiro orifício).
Fig. 5 – LCCA, male, 9 years old. Device stabilizing osteosynthesis. Start of plate sliding (first hole appears).
We are afraid to replace the stabilization method with external fixators, given its complications, both due to degenerative muscle injuries, trophic and functional changes they cause, and the risk of infection in patients undergoing chemotherapy. To resolve that situation, we requested the manufacture of a device consisting of two pieces of stainless steel that could be adapted to the proximal segment of the osteosynthesis, in order to maintain the support provided by the angled plate and at the same time allow it to slide and not block the bone growth (fig. 3). We performed a small medial surgical access, at the proximal end, removed the screws fixing the plate stem to the bone (directed from medial to lateral), and placed the curved blade between the femur and the plate, affixed the second blade shaped to adapt We placed it over the rod of the angled plate and screwed it in the anteroposterior direction (fig. 4).
Fig. 6 –Evidência de crescimento do osso e deslizamento da placa. Aparece o segundo “espaço de parafuso” – espessamento do enxerto – membros equalizados
Fig. 6 – Evidence of bone growth and plate slippage. The second “screw space” appears – thickening of the graft – equalized limbs
Fig. 7 – Continua o crescimento (aparece o terceiro orifício de parafuso). O fêmur operado cresceu mais que o outro lado. Escanograma confirmando.
Fig. 7 – Growth continues (third screw hole appears). The operated femur grew more than the other side. Scanogram confirming.
Fig. 8 – Pós-operatório de oito meses da primeira cirurgia (carga parcial) – joelhos desnivelados (maior à direita), carga total (1 ano e 1 mês da 2ª cirurgia).
Fig. 8 – Eight months post-operative period after the first surgery (partial weight-bearing) – unlevel knees (larger on the right), full weight-bearing (1 year and 1 month after the 2nd surgery).
In this way, we obtained good stability in the sense of blocking the efforts of rotational, varus, valgus, retrocurvatum or antecurvatum movements, but allowing the plate stem to slide as bone growth occurred (fig. 5).
Fig. 9 – R.N.M. determinando nível da ressecção, sacrificando a placa de crescimento da tíbia. Detalhe do periósteo recobrindo a lesão, dissecção do tendão patelar e músculo tibial anterior. Placa especial confeccionada e modelada para o paciente e dispositivo ocluído no extremo distal, com a aba de fixação angulada para adaptar-se ao formato triangular da tíbia.
Fig. 9 – MRI determining the level of resection, sacrificing the tibial growth plate. Detail of the periosteum covering the injury, dissection of the patellar tendon and anterior tibialis muscle. Special plate made and shaped for the patient and device occluded at the distal end, with the fixation flap angled to adapt to the triangular shape of the tibia.
In the fourth month postoperatively, after the placement of the extensible device (second surgery), we were able to verify the growth spurt and the sliding of the angled plate rod (fig. 6), at a distance of approximately one “screw space” ( fig. 5). The patient began partial weight bearing, walking with the aid of axillary crutches (fig. 5). In the control radiograph one year after placement of the device for extensible internal fixation, we were able to observe the continued sliding (fig. 6) of the angled plate rod, in which we visualized the advancement of yet another “screw space” (fig. 6). The fibula becomes thicker (fig. 6) and the patient increases weight on the operated limb (fig. 6). In the thirteenth month the third hole in the plate begins to appear (fig. 7). During the clinical examination of the patient (fig. 7), we were able to observe that the operated side grew two centimeters more than the non-operated side, confirmed by scanometry (fig. 7). This greater growth was due to the stimuli caused by the first surgery, the vascularized graft and the second surgery (placement of the extensible device). We observed that there has been an equalization in the size of members and we believe that at the end of the growth the members will be the same size or the difference will be minimal. The patient began to walk with full load (and 2cm compensation), and the longer operated side can be seen, where it can be seen that the knee level is lower on the operated side (fig. 8).
Fig. 10 – Emprego da fíbula proximal, com sua placa de crescimento, detalhe da inclinação em valgo do planalto tibial. Paciente com carga parcial, detalhe clínico do joelho em valgo. RX após 14 meses, com crescimento de 0,75cm pela placa fisária da fíbula e correção da angulação do planalto tibial. Paciente com carga e com correção clínica do valgo.
Fig. 10 – Use of the proximal fibula, with its growth plate, detail of the valgus inclination of the tibial plateau. Patient with partial weight bearing, clinical detail of the knee in valgus. X-ray after 14 months, with growth of 0.75cm through the fibular physeal plate and correction of the angulation of the tibial plateau. Patient with weight bearing and clinical valgus correction.
Fig. 11 – W.R.C., 15 anos, PO 18 meses. Paciente em crescimento, membros equalizados e alinhados. Boa função do joelho.
Fig. 11 – WRC, 15 years, PO 18 months. Patient growing, limbs equalized and aligned. Good knee function.
Fig. 12 – W.R.C., 16 anos. Pós-operatório 21 meses. Radiografia mostrando o fechamento da linha epifisária. Patiente com carga total e flexão do joelho.
Fig. 12 – WRC, 16 years old. Post-operative 21 months. Radiograph showing closure of the epiphyseal line. Patient with full load and knee flexion.

We currently use this device for immediately extensible internal fixation, and it currently consists of a single piece that has a curved side flap to adapt to the femur and humerus or it can be flat with an angle to adapt to the shape. triangular shape of the tibia (fig. 9). Another patient, WRC, 14 years old, with osteosarcoma of the right tibia, is a relatively recent example of reconstruction of the proximal metaphyseal segment of the tibia, with resection that also included the growth plate; We performed reconstruction with the fibula, including its epiphysis and using the epiphyseal plate of this fibula to provide growth (fig. 9). We can observe a slip of 0.75 cm by comparing the distances between the physeal plate of the transported fibula and the limit of the extensible device. Radiographic correction of the valgus inclination of the tibial plateau and clinical realignment of the knee can be seen (fig. 10). The patient is currently in the final phase of growth and has good knee function (Figs. 11 and 12).

COMMENTS

We believe that this device for extensible internal fixation, which we developed, can be used both for the treatment of selected cases of aggressive tumor lesions and also for other conditions, such as congenital malformations and sequelae of trauma or infections, which may require reconstructions that require a stabilization mechanism that allows the epiphysis to be fixed, but without blocking bone growth.

REFERENCES

1. Manfrini M., Gasbarrini A., Malaguti C., et al: Intraepiphyseal resection of the proximal tibia and its impact on lower limb growth. Clin Orthop 358: 111-119, 1999. 2. Eckardt JJ, Kabo JM, Kelley CM, et al: Expandable endoprosthesis reconstruction in skeletally immature patients with tumors. Clin Orthop 373: 51-61, 2000. 3. Capanna R., Bufalini C., Campanacci M.: A new technique for reconstructions of large metadiaphyseal bone defects. Orthop Traumatol 2:159-177, 1993. 4. Cool WP, Carter SR, Grimer RJ, Tillman RM, Walker PS: Growth after extendible endoprosthetic replacement of the distal femur. J Bone Joint Surg [Br] 79: 938-942, 1997. 5. Baptista PPR, Guedes A., Reggiani R., Lavieri RF, Lopes JAS: Tibialization of the distal fibula with preservation of the epiphyseal plate: preliminary report. Rev Bras Ortop 33: 841-846, 1998. 6. Baptista PPR, Guedes A., Reggiani R., Lavieri RF, Pires CEF: Tibialization of the fibula: description of the surgical approach. Rev Bras Ortop 33: 861-866, 1998.

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Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

Phone: +55 11 3231-4638  Cell:+55  11 99863-5577   Email:  drpprb@gmail.com

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Tibialization of the distal fibula

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Tibialization of the distal fibula. The authors describe the treatment of a nine-year-old child with osteosarcoma of the distal third of the right tibia, treated with neo-adjuvant chemotherapy and subsequently undergoing resection of the affected bone segment.

Tibialization of the distal fibula with preservation of the epiphyseal plate

Reconstruction was performed by arthrodesing the ipsilateral distal fibular epiphysis with the talus, preserving the fibular epiphyseal plate. Preliminary postoperative evaluation using axial slice scintigraphy demonstrated signs of capture of the fibula throughout the transposed extension and at the level of the projection of the distal fibular growth plate. It is not yet possible to distinguish the hypercapture of the physeal plate from the reparative process of arthrodesis at the level of the talus. Radiographic controls from September/98, nine months after surgery, show complete integration of the transposed fibula, both proximally and distally. Thickening of the fibula is already evident and the fibular growth plate can be easily distinguished.

INTRODUCTION: Osteosarcoma of the tibia in a child   

Osteosarcoma is the most common primary malignant bone tumor between the first and second decades of life(21). It generally affects the metaphyses of long bones, with the most common locations being the distal third of the femur and the proximal third of the tibia. Location in the distal third of the tibia represents approximately 3% of cases.

With the evolution of chemotherapy treatment, there was new encouragement in the approach to this condition, as it provided an increase in the average survival rate(2,7,19,21,22). This fact led to better improvements in the surgical techniques used until then. Malignant neoplasms previously treated with radical surgery, currently, when they respond favorably to neo-adjuvant chemotherapy, are approached with the aim of preserving the involved limb, with or without a biological solution(1,2,4,8,13,14,22). This concept has expanded, raising the expectations of the surgeon who seeks to combine the preservation of the affected body segment with the maintenance of maximum function(4,8,13).

Figura1 A: Radiografia frente do tornozelo direito, com lesão meta-epifisaria da tíbia, com limites imprecisos e triângulo de Codmann.
Figure 1 A: Front radiograph of the right ankle, with a meta-epiphyseal lesion of the tibia, with imprecise limits and Codmann's triangle.
Figura 1 B: Radiografia perfil do tornozelo direito, com osteossarcoma da região distal da tíbia.
Figure 1 B: Profile x-ray of the right ankle, with osteosarcoma of the distal region of the tibia.
Figura 1 C: Radiografia frente de osteossarcoma do tornozelo direito, em quimioterapia neo-adjuvante.
Figure 1 C: Frontal radiograph of osteosarcoma of the right ankle, undergoing neo-adjuvant chemotherapy.
Figura 1 D: Radiografia em perfil de osteossarcoma do tornozelo, em quimioterapia.pré-operatória.
Figure 1 D: Lateral radiograph of osteosarcoma of the ankle, undergoing preoperative chemotherapy.

CASE REPORT 

Female child, nine years and three months old, with a history of direct trauma to the right ankle two months ago, evolving with pain and local edema. She sought medical treatment and was diagnosed with a contusion. Plaster immobilization was performed for six days. Fifteen days later, she noticed an increase in volume in her ankle, which was painful and hardened, and sought our service. The radiograph revealed a radiolucent lesion, centrally located, in the distal third of the tibia, with imprecise radiographic limits and a thin laminar periosteal reaction (fig. 1). Bone scintigraphy showed intense uptake only at the site and nuclear magnetic resonance (fig. 2) showed intense involvement of the meta-epiphyseal region, with evident involvement of the tibial epiphyseal plate. Laboratory tests demonstrated changes in bone metabolism, with very high alkaline phosphatase and serum calcium. We performed a needle biopsy, and the diagnosis of chondroblastic osteosarcoma was confirmed. Neo-adjuvant chemotherapy treatment began, with three cycles of chemotherapy. As part of the pre-operative surgical planning, we performed arteriography (fig. 3) to visualize the emergence of the nutrient artery of the fibula, a time that we considered important to identify the safe site for the osteotomy and its transposition. A polyethylene cruropodal orthosis was made prior to surgery, aiming for adequate immobilization, providing better support for the limb in the postoperative period (fig. 4). After neo-adjuvant chemotherapy, she underwent surgical treatment.

Fig. 3 Arteriografia pré-operatória
Fig. 3 Preoperative arteriography
Fig. 4 – Órtese confeccionada antes da cirurgia
Fig. 4 – Orthosis made before surgery
Fig. 5 – Ressecção do tumor com margem oncológica
Fig. 6 Osteotomia da fise, mantendo-se a fise distal. Observa-se a cruentização da epífise fibular e permanência de um segmento do maléolo medial.
Fig. 5 – Resection of the tumor with oncological margin
Fig. 6 Osteotomy of the physis, maintaining the distal physis. Opening of the fibular epiphysis and permanence of a segment of the medial malleolus are observed.

DESCRIPTION OF OPERATIVE TECHNIQUE

The surgery is performed with a medial convex arcuate incision starting at the level of the head of the fibula, passing through the anterior surface of the leg, up to the end of the lateral malleolus. The lesion is resected with a macroscopic oncological margin in the soft tissues and a 3.0 cm bone margin (fig. 5). After resection of the tumor, using the interosseous membrane as a guide, we approached the site of the osteotomy of the proximal fibula, above the emergence of the nutrient artery, confirmed by a previous arteriographic study (fig. 6). In this case, as the resection of the tibial segment was smaller, we opened a slit on the lateral surface of the proximal segment of the tibia, approximately 3.5 cm long and wide enough to enable its interlocking, with minimal deperiostization. from the proximal end of the transposed segment, and without harming the nutrition provided by the nutrient artery. Next, we remove the cartilage from the fibular epiphysis and carve a hole in the dome (fig. 7) of the talus, allowing this distal fibular epiphysis to fit. We continued with the careful passage of a 2.5mm diameter wire through the medullary canal of the fibula, crossing the physeal plate. This thread continues through the epiphysis and is passed through the talus and calcaneus until it appears on the skin (fig. 8).

Fig. 7 – Escavação no domo do tálus
Fig. 7 – Excavation in the talus dome
Fig. 8 – Passagem do fio longitudinalmente pelo calcâneo, tálus, fíbula transposta e tíbia
Fig. 8 – Passing the wire longitudinally through the calcaneus, talus, transposed fibula and tibia
Fig. 9 – Redução dos fragmentos
Fig. 9 – Reduction of fragments
We proceeded with nailing the proximal end of the fibula segment into the bed prepared on the tibia. The steel wire is then passed in a retrograde direction, through the medullary canal of the tibia, to the metaphyseal region (fig. 9). The tibial malleolus was arthrodesed to the talus and fixed with a 1.5 mm diameter wire, passing through the talus and crossing the epiphyseal segment that was embedded in it. The stabilization of the proximal fragment was complemented by placing a bone pin between the fibula and the tibia, within the tibial medullary canal, in order to prevent the fibula from migrating proximally, as there is a discrepancy in diameter between the ends of the fragments. This pin was obtained from the tibia itself, during the preparation of the bed for the placement of the fibular segment (fig. 10).
Figura 10 A: Radiografia frente, em 1998.
Figure 10 A: Front radiograph, in 1998.
Figura 10 B: Radiografia perfil, em 1998.
Figure 10 B: Profile x-ray, in 1998.
Figura 10 C: Radiografia frente, após um ano da cirurgia. Fíbula integrada e já tibializada com significativo espessamento cortical.
Figure 10 C: Front radiograph, one year after surgery. Fibula integrated and already tibialized with significant cortical thickening.
Figura 10 D: Radiografia perfil, após um ano da cirurgia. Fíbula já tibializada
Figure 10 D: Profile x-ray, one year after surgery. Fibula already tibialized
Figura 11 A: Cintilografia óssea, fase tardia, mostrando captação no 1/3 distal da perna, confirmando a presença de vascularização da fíbula transplantada.
Figure 11 A: Bone scintigraphy, late phase, showing uptake in the distal 1/3 of the leg, confirming the presence of vascularization of the transplanted fibula.
Figura 11 B: Cintilografia óssea da perna destacando a captação da fíbula e a ossificação do trajeto da transferência proximal, promovendo uma fusão proximal.
Figure 11 B: Bone scintigraphy of the leg highlighting capture of the fibula and ossification of the proximal transfer path, promoting proximal fusion.
Fig. 12 – Radiografias tiradas no pós-operatório de nove meses
Fig. 12 – Radiographs taken nine months postoperatively

In the immediate postoperative period, the limb was kept in immobilization with the previously made cruropodal device. Six weeks after surgery, we performed bone scintigraphy with axial sections, confirming good vascularization of the graft (fig. 11). In the metaphyseal region of the transplanted fibula, the increase in hyperuptake may be due to the vascularization of the physeal plate itself and also to the reparative process at the site of the talofibular arthrodesis. Radiographic controls from September/98, nine months after surgery, show complete integration of the transposed fibula, both proximally and distally. The thickening of the fibula is already evident and its growth plate can be easily distinguished (fig. 12).

DISCUSSION

Advances in polychemotherapy in the treatment of osteosarcoma have brought new perspectives regarding the prognosis and approach of affected patients. Controlling the disease through chemotherapy made it possible to preserve limbs, allowing new possibilities and the most varied solutions to be proposed(4,8,13). One of the solutions was the replacement of the affected segment with non-conventional internal prostheses. However, in young children, there are basically two major problems: patients continue to grow and prostheses become insufficient, making amputation necessary in some cases, often years after the start of treatment(5 .6); Furthermore, prostheses suffer excessive wear and young patients have to undergo early revisions. Prosthetics in children have very limited indications(6). With the considerable increase in survival, it became necessary for the orthopedic surgeon to look for long-lasting limb-saving surgical solutions. The use of a homologous graft to fill the bone gap is an option. However, in addition to the greater difficulty in integrating the graft, the greater number of complications, even exceeding those of endoprostheses, it also requires a bone bank, which is not always possible in our reality(6). The use of autologous grafts is sometimes limited when there is a need to replace large resections. Vascularized bone grafting has been used more frequently and presents good results. When replacing segments of the tibia, the option has been to use the ipsilateral or contralateral vascularized fibula, and several techniques have been proposed(3,5,9-12,15-18,23-25). Some of these techniques are performed in two operative stages, which increases morbidity. Microsurgical techniques are also used, but they require a specialized team, with prolonged surgical time. The technique presented here is quick, easy to perform, performed in a single surgical procedure and does not require a microsurgical technique. In an attempt to preserve the length of the limb, we transposed a segment of the fibula with the distal physis, hoping that it will remain active. We cannot yet say, due to the short follow-up period, that preservation of the physis in the fibular transposition technique will lead to bone growth, nor how this growth will occur.

PRELIMINARY CONCLUSIONS

The biological solution in the treatment of osteosarcoma is an increasingly common reality in our country and should always be considered. We believe that the presence of bone hyperuptake at the level of the projection of the physeal plate of the distal fibula in bone mapping exams may be evidence that it is viable, although it is impossible to distinguish how much of this process is due to bone reaction at the level of fixation of the epiphysis of the fibula in the body of the talus. Considering the short follow-up period and the fact that this is a single case, it is not possible to definitively evaluate the treatment method used. What we can say with satisfaction, at the moment, is that the radiographic controls from September/98, nine months after surgery, show complete integration of the transposed fibula, both proximally and distally. Thickening of the fibula is already evident and the fibular growth plate can be easily distinguished. We believe that growth will occur and we hope that there will be adaptation of this physeal plate, so that it grows with the speed of the tibia, as we know that the speed of growth is also influenced by the location in which it is located.

REFERENCES:

1. Bacci, G. et al: Primary chemotherapy and delayed surgery for non-metastatic telangiectasic osteosarcoma of the extremities: results in 28 patients. Eur J Cancer 30A: 620-626, 1994.

2. Campanacci, M.: “Classic osteosarcoma”, in Campanacci, M. et al: Bone and soft tissue tumors, Bologna, Aulo Gaggi Ed., 1990. p. 455-480.

3. Campbell, WC: Transference of the fibula as an adjunct to free bone graft tibial deficiency: report of three cases. J Orthop Surg 1:625, 1919.

4. Carter, SR, Grimer, RJ & Sneath, RS: A review of 13-year experience of osteosarcoma. Clin Orthop 270: 45-51, 1991.

5. Chacha, PB: Vascular pedicle graft of the ipsilateral fibula for non-union of the tibia with a large defect. J Bone Joint Surg [Br] 63: 244- 253, 1981.

6. David, A. et al: Osteosarcoma: review of 39 cases. Rev Bras Ortop 33: 45-48, 1998.

7. Davis, AM, Bell, RS & Goodwin, PJ: Prognostic factors in osteosarcoma: a critical review. J Clin Oncol 12: 423-431, 1994.

8. Dubousset, J., Missenard, G. & Kalifa, C.: Management of osteogenic sarcoma in children and adolescents. Clin Orthop 270: 52-59, 1991.

9. Girdlestone, GR & Foley, WB: Extensive loss of tibial diaphysis. Ti-bio-fibular grafting. Br J Surg 20: 467-471, 1933.

10. Hahn, E.: Eine methode, pseudoarthrosen der tibia mit grossem knochen defect zur heitung zubringen. Zentralbl Chir 11: 337-341, 1884.

11. Huntington, TW: Case of bone transfer. Ann Surg 41: 249-251, 1905.

12. Jones, KG & Barnett, HC: Cancellous bone grafting for non-union of the tibia through the posterolateral approach. J Bone Joint Surg [Am] 37: 1250-1260, 1955.

13. Lane et al.: Osteogenic sarcoma. Clin Orthop 204: 93-110, 1986.

14. Marwin, MR: Amputation for osteosarcoma. Cancer Bull 42: 337-343, 1990.

15. McCarrol, HR: The surgical management of ununited fractures of the tibia. JAMA 175: 578-583, 1961.

16. McMaster, PE & Hohl, M.: Tibiofibular cross-peg grafting. J Bone Joint Surg [Am] 47: 1146-1158, 1965.

17. Meyerding, HW: Tibial defects with non-union created by the transfer of the fibula and tibiofibular fusion. Am J Surg 52:397-404, 1941.

18. Milch, H.: Synostosis operation of persistent non-union of the tibia. A case report. J Bone Joint Surg [Am] 21: 409-420, 1939.

19. Petrilli, S. et al: IIB osteosarcoma. Current management, local control and survival statistics – São Paulo, Brazil. Clin Orthop 270: 60-66, 1991.

20. Picci et al: Relationship of chemotherapy-induced necrosis and surgical margins to local recurrence in osteosarcoma. J Clin Oncol 12: 2699-2705, 1994.

21. Simon, AM & Springfield, D.: Surgery for bone and soft tissue tumors, Baltimore, Lippincott-Raven, 1998. p. 266.

22. Spanier, SS, Shuster, JJ & Griend, RAV: The effect of local extent of the tumor on prognosis in osteosarcoma. J Bone Joint Surg [Am] 72: 643-653, 1990.

23. Stone, JS: Partial loss of the tibial replaced by transfer of the fibula, with maintenance of both malleoli of the ankle. Ann Surg 46: 628-632, 1907.

24. Taylor, GI & Millar, GDH: The free vascularized bone graft, a clinical extension of microvascular techniques. Plast Reconstr Surg 55: 533-544, 1975.

25. Wilson, PO: A simple method of two-stage transplantation of the fibula for use in cases of complicated and congenital pseudoarthrosis of the tibia. J Bone Joint Surg [Am] 23: 639-675, 1941.

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Author: Prof. Dr. Pedro Péricles Ribeiro Baptista

 Orthopedic Oncosurgery at the Dr. Arnaldo Vieira de Carvalho Cancer Institute

Office :  Rua General Jardim, 846 – Cj 41 – Cep: 01223-010 Higienópolis São Paulo – SP

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