Tumorous Diseases in Patients with the Testicular Feminization Syndrome
(„Androgen Insensitivity” Syndrome) - Description of Two Cases
Hes O.1, Vaněček T.1, Šíma R.1, Hora M.2, Veličkinová H.2, Grossmann P.1, Kovář J.3, Michal M.1
1Oddělení speciální diagnostiky ŠPAÚ, FN Plzeň, přednosta prof. MUDr. M. Michal 2Urologická klinika, FN Plzeň, přednosta doc. MUDr. M. Hora, PhD. 3Oddělení gynekologie a porodní, NsP Domažlice, primář MUDr. J. Kovář |
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Summary:
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
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