Clear cell sarcoma is a rare tumor with a predilection to involve tendon and aponeuroses, predominantly around the foot and ankle region. It is clinically, genetically as well as biologically distinct from malignant melanoma, however share some histological and immunohistochemical similarities with it. Clear cell sarcoma usually arise from the deep soft tissue of lower extremities and carries a balanced translocation t(12;22) (q13;q12). The fusion protein activates MiTF, a melanocytic transcription factor which leads to tumor growth and melanin production. The head & neck and trunk are distinctly unusual sites for occurrence of CCS. We herewith describe a CCS in the pelvis of the 26 years-old male patient presented with the chief complaints of paraesthesia, weakness, muscle wasting in right lower limb with foot drop since one year with review of literature.
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