Clear Cell Sarcoma With Cutaneous Presentation in a 4-Year-Old Boy

We report a case of a 4-year-old Brazilian boy, who presented with an erythematous and painful nodule involving the skin of his left arm. Immunohistochemistry was performed for S100, SOX10, CD34, desmin, SMA, HMB-45, CD1a, and CD163, and fluorescence in situ hybridization for EWSR1 gene rearrangement using a break-apart probe was completed. Immunohistochemistry showed bland spindle cells with "floret-like" appearance simulating a giant cell fibroblastoma; tumor cells were positive for S100 and SOX10; neoplastic cells were negative for CD34, desmin, SMA, HMB-45, CD1a, and CD163; and fluorescence in situ hybridization showed an EWSR1 gene rearrangement. We report the youngest known case of cutaneous involvement of clear cell carcinoma at the age of 4.