title: Predicting the Behavior of Perivascular Epithelioid Cell Tumors of the Uterine Corpus.
abstract: Perivascular epithelioid cell tumors (PEComas) are rare neoplasms that share phenotypic features with angiomyolipomas, clear cell sugar tumors, and lymphangioleiomyomatosis. They presumably represent the neoplastic counterpart of a yet-unidentified perivascular epithelioid cell that expresses smooth muscle and melanocytic immunomarkers. The uterus is the second most common site of origin for perivascular epithelioid cell tumors, after the retroperitoneum. Although most uterine perivascular epithelioid cell tumors are clinically benign and can be cured by a complete surgical excision, there is a subset characterized by both local and distant dissemination. Unfortunately, no single histopathologic or immunohistochemical parameter can accurately predict the clinical behavior of these tumors, which is why the 2012 World Health Organization classification of tumors of the female reproductive organs suggests the use of several criteria to predict the risk of aggressive clinical behavior. Here we review those perivascular epithelioid cell tumors of the uterine corpus with aggressive clinical behavior reported in the literature, and we discuss their most relevant clinical and histopathologic features.
authors.forename: Andres Martin
authors.initials: AM
authors.lastname: Acosta
authors.affiliation: From the Department of Anatomic/Clinical Pathology, University of Illinois at Chicago Hospital and Health Sciences System (Dr Acosta); and the Department of Pathology, Advocate Lutheran General Hospital, Park Ridge, Illinois (Dr Adley).
authors.forename: Brian P
authors.initials: BP
authors.lastname: Adley
mesh_terms: D005260:Female; D006801:Humans; D054973:Perivascular Epithelioid Cell Neoplasms / Q000473:pathology*; D014594:Uterine Neoplasms / Q000473:pathology*