Malignant perivascular epithelioid cell tumor of the uterus

Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors arising in a wide array of anatomic locations and characterized by a myomelanocytic phenotype. PEComas which occur in non - classic anatomic distributions are known as perivascular epithelioid cell tumor - not otherwise specified (PEComa - NOS), and one of the most common primary sites for PEComa - NOS is the uterus. The risk of aggressive behavior of these tumors has been linked to a number of factors evaluable on pathologic review following initial surgical resection. We report a case of PEComa - NOS of the uterus with multiple high - risk features, including frank vascular invasion, with no evidence of recurrent disease 18 months following initial surgical resection.