Perivascular Epithelioid Cell Tumor of the Buttock Region

Perivascular epithelioid cell neoplasms, also known as PEComas, are a group of rare mesenchymal tumors that have a perivascular distribution and have no known counterpart to normal cells. The PEComa grouping includes angiomyolipomas, lymphangioleiomyomatoses, clear cell (sugar) tumors at extrapulmonary and intrapulmonary sites, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres among others. These rare tumors most commonly arise in the uterus. Here, we present an unusual case of malignant PEComa arising in the buttock region.