Clear cell sarcoma is a rare tumor and accounts for just over 2% on all childhood non-RMS. Also known as malignant melanoma of the soft parts.
This tumor has a characteristic translocation t(12;22)(q13;q12)
This tumor presents as a slowly growing soft tissue mass in extremities (often the foot or ankle) which generally grows in association with tendons and aponeurosis.
Most common in young adults (median age was 15 years at presentation in one study)
Pathology shows:
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Nests or short fascicles of spindle shaped or epithelioid cells with abundant clear to granular eosinophilic cytoplasm separated by fibrous septa
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Surrounds normal epithelial elements
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Also multinucleated giant cells
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Variable melanin
CCS is associated with a significant risk of lymph node metastases
Complete surgical resection with negative margins is the most effective therapy.
Chemotherapy has not been shown to be effective in this disease.
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