Clear cell sarcoma or Malignant melanoma of soft part - a rare entity

Clear cell sarcoma is an extremely rare, slow growing and aggressive soft tissue sarcoma with melanocytic differentiation. We report a case of recurrent clear cell sarcoma treated at our Institute for its rarity and need for appropriate diagnosis and treatment. A 26 year old male presented to our Institute with recurrent swelling in the right popliteal fossa for 1 month duration. He had previous excision of the right popliteal fossa lesion and reported as alveolar rhabdomyosarcoma outside and he came to our Institute for further treatment. On evaluation he was diagnosed with non-metastatic recurrent clear cell sarcoma of the right popliteal fossa. He had right popliteal fossa exploration and underwent right above knee amputation in view of popliteal vessel and nerve involvement. He had an uneventful recovery and is receiving ifosfamide and doxorubicin based adjuvant chemotherapy. Clear cell sarcoma is a rare melanin producing soft tissue sarcoma with diagnostic challenge. Complete surgical excision is the optimal treatment for local control.