Bb4b90201e39e55c4a9ccfec8436cfb8 3
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 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.
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 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 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 21: Apresentando baixo sinal em T1.
Figure 20: Soft tissue chondrosarcoma of the hand. Figure 21: Showing low signal on 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 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 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 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 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 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 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 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%  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 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 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 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 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 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 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 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 76: A arteriografia revela hipervascularização.
Figure 75: Hyperuptake on bone scintigraphy, suggesting increased metabolic activity. Figure 76: Arteriography reveals hypervascularization.
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 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 84: TC com janela para tecidos moles.
Figure 83: CT with bone window. Figure 84: CT with soft tissue window.
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 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 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 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 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

Deixe um comentário

Olá! Como podemos auxiliá-lo?