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