Cutaneous PEComa: a rare entity to consider in an unusual site.

PEComa is a mesenchymal neoplasm thought to derive from the perivascular epithelioid cell, a hypothetical cell type not yet identified. PEComa is a broad term that was recently expanded to include visceral, retroperitoneal, and somatic soft tissue, in addition to cutaneous tumors, which share morphological and immunohistochemical features in common with angiomyolipoma, lymphangiomyomatosis, and clear cell "sugar" tumor. The latter have distinct, site-related, clinical, morphological, and biological features. PEComas share features of concurrent melanocytic and myocytic differentiation. Most cases are sporadic, but renal and pulmonary PEComas can be part of the tuberous sclerosis syndrome more often than PEComas from other sites. We report a case of cutaneous PEComa to raise awareness of the entity occurring at this site and caution against misinterpretation as a primary or metastatic malignancy, for example melanoma or renal cell carcinoma, clear cell type.