Gynecology & Obstetrics
Open Access

Abstract

A Lymphatically Metastasized Perivascular Epithelioid Cell Tumor from the Uterus

Klerkx WM, Sie-Go DMDS, Daan NMP, Witteveen PO and Verheijen RHM

Perivascular Epithelioid tumor (PEComa) is a rare malignancy which may occur at various anatomic sites. A case is described of PEComa in multiple lymph nodes in a 34 year old woman with adenocarcinoma of the cervix. Laparoscopic pelvic lymph node dissection revealed perivascular epithelioid tumor cells in 15/34 pelvic lymph nodes and no sign of the adenocarcinoma. At subsequent radical hysterectomy the primary tumor was found in the uterus, besides the stage IB1 adenocarcinoma of the cervix. No adjuvant treatment was given and the patient remained well, until the time of evaluation, 20 months after diagnosis.

A systematic review is performed about gynecological PEComa with lymphatic involvement concerning diagnosis, treatment and overall survival.