Epithelioid sarcoma arising on the nose of a child: a case report and review of the literature

A 4-year-old boy presented with a 6-month history of a red papule on the na sal septum. Physical examination was otherwise unremarkable. A biopsy speci men showed an epithelioid sarcoma characterized by nodular collections of e pithelioid tumor cells with central, tumor cell necrosis. By immunohistoche mistry the tumor cells were positive for cytokeratin, epithelial membrane a ntigen, vimentin, and CD34, but negative for S-100, CD31, factor VIII-relat ed antigen, CD68, actin, desmin and myoglobin. Epithelioid sarcoma is an un common tumor of uncertain histogenesis that typically arises in the extremi ties of young adults. Both the age of our patient and the location of his t umor are unusual, emphasizing the spectrum of presentations that may occur with epithelioid sarcoma. Epithelioid sarcoma should be considered in the d ifferential diagnosis of granulomatous diseases and epithelioid tumors of c hildren, even in unusual locations.