| Summary: | Cutaneous clear cell sarcoma (CCS) is a rare soft tissue malignancy that typically manifests in the distal extremities of young adults. Although it shows melanocytic differentiation, CCS is clearly pathologically and genetically distinct from malignant melanoma. Here, we report the case of a 43-year-old male who had an asymptomatic, deep-seated, slowly enlarging, firm mass over the right heel for 30 years that recently and rapidly progressed with tenderness. We arranged for the total excision of the tumor. Pathological and cytogenetic analysis of the biopsied specimen showed that it was a clear cell sarcoma. Computed tomography and positron emission tomography scans showed no signs of metastasis, and no other abnormal hypermetabolic lesions were detected. Wide excision with split-thickness skin graft and a sentinel lymph node biopsy were performed. Because of positive findings in the sentinel lymph nodes, the patient was transferred to the plastic surgery department for further radical popliteal and inguinal lymph node dissection. The patient has received regular outpatient follow-up care in our hospital for the past 8 months with no evidence of recurrence.
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