Clear cell chondrosarcoma: a review of clinicopathologic characteristics, differential diagnoses, and patient management.

Clear cell chondrosarcoma (CCC), an extremely rare primary bone tumor, is currently classified by the World Health Organization as a low-grade malignant cartilaginous neoplasm. Clinically, CCC occurs primarily in males with a peak incidence in the third to fifth decades of life, and occasionally, it presents in skeletally immature patients. Unlike conventional chondrosarcoma, CCC has a predilection for the epiphysis of long bones and often displays radiologic features reminiscent of chondroblastoma. The recommended treatment is wide operative resection. CCC has a local recurrence rate of approximately 30%, and nearly 20% cases metastasize mainly to bone and lung often a decade after surgical intervention. Incomplete excision or curettage is associated with a high rate of recurrence. Histologically, the process is characterized by infiltrative lobules and sheets of round to oval cells with abundant cleared cytoplasm and well-defined cell borders associated with trabecula of osteoid and woven bone, scattered osteoclasts, and foci of conventional low-grade chondrosarcoma in about one-half of cases. Correlation with clinical and radiologic characteristics, such as epiphyseal location and young patient age, assists in establishing a correct diagnosis. Pathologic diagnosis of CCC is complicated by the low diagnostic accuracy of core needle biopsy, overlapping histologic features with other matrix-rich primary bone tumors, and a lack of a specific immunohistochemical and molecular profile. DNA methylation-based profiling classifier (sarcoma classifier) is one recent technologic advancement that may help to confirm the histopathological diagnosis of CCC or indicate the need for thorough reassessment in cases where results contradict previous conventional findings.

Clear cell chondrosarcoma of proximal femur: a rare entity with diagnostic dilemma.

Clear cell chondrosarcoma is an extremely rare malignant neoplasm. The rarity and slow-growing nature of this tumour often lead to prolonged symptoms and also initial misdiagnosis with benign lesions such as chondroblastoma. It can also be confused with avascular necrosis of the femoral head when the lesion is located in the femoral head, as was in the case we report. The patient was kept on observation and conservative treatment for almost 9 years before the correct diagnosis and appropriate treatment. Wide local resection with negative margins forms the mainstay of treatment since intralesional procedures predispose to high local recurrence rate. A prolonged follow-up is recommended since late local recurrences and metastases are common.

Clear Cell Chondrosarcoma With Chondroblastoma-Like Features: A Case for Team Diagnosis.

Clear cell chondrosarcoma (CCCS) is a rare variant of conventional chondrosarcoma with low-grade malignant features that may be confused radiographically and histologically with chondroblastoma. We report a case of a 50-year-old female who presented with 6 months of left hip pain. Initial radiographs demonstrated an osteolytic lesion with adjacent area of sclerosis in the proximal left femur. Magnetic resonance imaging demonstrated a marrow-infiltrative lesion with periosteal reaction and thickened enhancing periosteum. Biopsy of the sclerotic area demonstrated chondroblastoma-like findings, whereas biopsy of the lytic area showed features suggestive of CCCS. The patient eventually underwent en bloc resection and reconstruction with a proximal femoral megaprosthesis. The final diagnosis was CCCS. We present this unusual case with review of the radiographic and histologic features of CCCS with attention to its ability to mimic chondroblastomas. This case highlights the importance of sampling radiographically heterogeneous areas within a bone lesion to facilitate accurate diagnosis and appropriate management.