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Case Reports in Obstetrics and Gynecology
Volume 2017, Article ID 8357235, 4 pages
https://doi.org/10.1155/2017/8357235
Case Report

Bilateral Sertoli Cell Tumors in a Patient with Androgen Insensitivity Syndrome

1Department of Obstetrics and Gynecology, Santa Casa de Misericórdia, Vitória, ES, Brazil
2Santa Casa de Misericórdia, Vitória, ES, Brazil

Correspondence should be addressed to Roberta Fonseca de Souza; moc.liamg@2070acesnofatrebor

Received 19 September 2016; Accepted 27 February 2017; Published 13 March 2017

Academic Editor: Hans Rudolf Tinneberg

Copyright © 2017 Roberta Fonseca de Souza et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Androgen insensitivity syndrome is the most common cause of male pseudohermaphroditism and the third most common cause of primary amenorrhea. This genetic alteration is a consequence of inherited defects on the X chromosome causing total or partial damage to the intrauterine virilization process due to functional abnormalities in the androgen receptors. The present report describes a 22-year-old patient with a female phenotype and a 46, XY karyotype, presenting with bilateral inguinal tumors. The tumors were surgically removed at the Santa Casa de Misericórdia Hospital in Vitória, Espírito Santo, Brazil. Pathology revealed bilateral testicles with Sertoli cell tumors. According to the international literature, prophylactic gonadectomy following puberty is recommended due to the progressive risk of neoplastic transformation in the residual gonads.