Perivascular epithelioid cell tumor of abdominal origin

de León, David Cantú; Pérez-Montiel, Delia; Bandera, Arfy; Villegas, Carlos; Gonzalez-Conde, Esther; Vilchis, José Chanona

doi:10.1016/j.anndiagpath.2010.02.007

« Previous Next »Annals of Diagnostic Pathology
Volume 14, Issue 3 , Pages 173-177, June 2010

Perivascular epithelioid cell tumor of abdominal origin

David Cantú de León, MD
- Department of Gynecologic Oncology, Instituto Nacional de Cancerología, México City, México
Delia Pérez-Montiel, MD
- Department of Pathology, Instituto Nacional de Cancerología de México, México City 14080, México
- Corresponding author. Instituto Nacional de Cancerologia, México City 14080, México. Tel.: +52 55 32 44 31 87; fax: +52 55 55 73 70 37.
Arfy Bandera, MD
- Department of Gynecologic Oncology, Instituto Nacional de Cancerología, México City, México
Carlos Villegas, MD
- Department of Gynecologic Oncology, Instituto Nacional de Cancerología, México City, México
Esther Gonzalez-Conde, MD
- Department of Pathology, Centro Oncológico de Tamaulipas, Secretaria de Salud
José Chanona Vilchis, MD
- Department of Pathology, Instituto Nacional de Cancerología de México, México City 14080, México

Abstract

Perivascular epithelioid tumor is not a common disease; therefore, large series are not available in the literature, and most are case reports. Histologic characteristics of these neoplasms are positivity to melanogenic (HMB-45) and muscle stains. All these neoplasms have the characteristic perivascular epithelioid cell or “PEC,” but the term myomelanocyte tumor has been proposed because of the immunohistochemistry expression. Females are affected more commonly than males. The uterus seems to be one of the most common organs affected. Its clinical behavior is uncertain, and standard treatment has not been established as well as criteria for malignancy. We report 3 cases of abdominal PEComa, 1 of them with benign clinical outcome and 2 with an aggressive behavior.

Keywords: PEComa, Malignant, Pelvis