Perivascular epithelioid cell tumor arising in the sacrum: a case report.

Abstract

Perivascular epithelioid cell tumor (PEComa) is a rare and recently described neoplasm composed mainly of epithelioid cells with a characteristic perivascular pattern of arrangement.1 The diagnosis can be confirmed by immunohistochemical (IHC) staining as tumor cells express melanocytic and myogenic markers.2 These tumors can occur in a wide variety of organs including the kidney, liver, lung, uterus, and pancreas.3 We report the case of a PEComa arising from the sacral bone, which is an exceedingly rare site for a PEComa. Although there have been several reports of PEComas originating from bone, to our knowledge this is the first case report of a PEComa arising from the sacral bone.4