Tumorous Diseases in Patients with the Testicular Feminization Syndrome („Androgen Insensitivity” Syndrome) - Description of Two Cases

Czech version

Authors: O. Hes ¹; T. Vaněček ¹; R. Šíma ¹; M. Hora ²; H. Veličkinová ²; P. Grossmann ¹; J. Kovář ³; M. Michal ¹
Authors‘ workplace: Oddělení speciální diagnostiky ŠPAÚ, FN Plzeň, přednosta prof. MUDr. M. Michal ¹; Urologická klinika, FN Plzeň, přednosta doc. MUDr. M. Hora, PhD. ²; Oddělení gynekologie a porodní, NsP Domažlice, primář MUDr. J. Kovář ³
Published in: Ceska Gynekol 2005; 70(2): 113-117
Category: Original Article

Overview

Objective:
To describe tumors occurring in two cases of testicular feminization syndrome.

Subject:
Case report.

Setting:
Dpt. of Special Diagnostics ŠPAÚ, University Hospital Plzeň

Subject and method:
Two cases of testicular feminization syndrome were selected from four cases in our registry. Patients were 45 and 84-year-old Caucasian „females“. Resected material was fixed in formaldehyde, routinely processed and stained with hematoxiline-eosin, inhibin, cytokeratines 20, placentar alkaline phosphatase, CD 99, Melan A, hCG. Sertoli cell adenoma was diagnosed in both patients. Older patient had in addition unclassified sex cord tumor of Leydig cell type. The number of sex chromosomes was examined using FISH analysis in both patients.

Conclusion:
Patients with testicular feminization syndrome are frequently affected by benign or malignant tumors in the cryptorchid testes. We documented two benign Sertoli cell adenomas and one sex cord tumor of uncertain biological behavior in our patients. The testes should be removed after puberty with subsequent estrogen therapy in patients with testicular feminization syndrome.

Key words:
androgen insensitivity syndrome, testicular feminization, tumors, Sertoli cell adenoma