Malignant perivascular epithelioid cell tumor of the uterus

Rare Tumors. 2012 Jan 2;4(1):e14. doi: 10.4081/rt.2012.e14. Epub 2012 Mar 12.


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.

Keywords: PEComa; perivascular epithelioid cell; uterine cancer..

Publication types

  • Case Reports