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Clear Cell Sarcoma : Prognosis

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Clear cell sarcoma is an aggressive tumor characterized by progressive disease, repeated local recurrences (15 to 40% of cases), and eventual distal metastases (lung, lymph nodes) in about 50% of cases. Metastases may develop more than 10 or 20 years after initial resection, necessitating long-term surveillance. Tumor size and the presence of necrosis are most important prognostic factors. The 5-yr., 10-yr., and 20-yr survival rates are 47-67%, 33%, and 10% respectively.

The image shows a clear cell sarcoma which focally resembled malignant peripheral nerve sheath tumor; however, there were clear-cut areas with conventional morphology of clear cell sarcoma. The diagnosis was confirmed by the presence of chromosomal translocation t(12;22)(q13;q12).

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