Malignant rhabdoid tumor of the vulva: A case report and review of the literature

Abstract

Malignant rhabdoid tumor was originally described in the kidney as a variant of Wilms' tumor in 1978 by Beckwith and Palmer [1]. Extrarenal forms of this tumor are very rare but have been described in a variety of sites including soft tissues, paraspinal and paratesticular areas, the central nervous system, colon, tongue, prostate, liver, skin, thymus, heart, pelvis, uterus, and vulva. Only 13 vulvar cases have been reported thus far in the English literature. We are reporting the 14th case and a review of the clinical features, management and prognosis of all cases. A 51-year-old G4P3, postmenopausal woman presented to our Obstetrics and Gynecology Clinic, Erciyes University Faculty of Medicine, Kayseri, Turkey with a painless vulvar mass measuring 3 cm that was situated on the left labium major adjacent to the anterior comissura. The lesion first appeared 2 months previously and was locally excised by a gynecologist at a secondary care center with the presumed diagnosis of lipoma. The histopathological examination revealed an extrarenal rhabdoid tumor (ERT). At the time of referral to our center, 20 days after the excision, a recurrent tumor measuring 4 cm, which was painless, with irregular border and arising from the excision site was detected (Figure 1). On transvaginal ultrasonography, the uterus, endometrial thickness, and both ovaries were normal. A positron emission tomography/ computer tomography with 18-F-fludeoxyglucose (18-F-FDG PET/CT) was performed for metastases screening. The 18-F-FDG PET/CT detected a soft-tissue lesion showing hypermetabolic