Perivascular Epithelioid Cell Tumor: A Rare Liver Primary of Mesenchymal Origin

Perivascular Epithelioid Cell Tumors (PEComas) are a family of rare neoplasms defined as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells [1]. Pea et al. first noted this unusual cell type in both renal angiomyolipoma (AML) and clear-cell “sugar” tumor (CCST) of the lung [2]. This generated the concept of a family of neoplasms composed of a distinctive cell type, which was immunoreactive with melanocytic markers, and exhibits an epithelioid appearance, a clearacidophilic cytoplasm, and a perivascular distribution [3]. Cases of primary PEComa of the liver are considered extremely rare [4]. Prior to 2011, approximately 100 cases of PEComa had been described originating from different sites but only few cases were reported of hepatic origin [5]. We present a case of liver PEComa in a young female initially diagnosed as an adenoma with an area of intra-tumoral hemorrhage.