The persistent mu¨llerian duct syndrome is a rare form of male intersex disorder in which a phenotypically normal male subject has fallopian tubes and a uterus along with testes and wolffian duct derivatives. This internal male pseudohermaphroditism is caused by a deficiency of mu¨llerian duct inhibitory factor.1 A genetic mutation of chromosome 19 seems to be implicated in this condition.2 Like other undescended testes these gonads are at increased risk of malignant transformation, and a variety of germ cell tumors have been reported in association with this syndrome.3 These patients often present with cryptorchidism or an inguinal hernia with fallopian tubes and a uterus. We report a case of bilateral synchronous germ cell tumor in a patient with the persistent mu¨llerian duct syndrome.
Bilateral testicular carcinoma in patient with the persistent mullerian ductsyndrome.
Bucci, Stefano;Liguori, Giovanni;BUTTAZZI, LORENZO;Bussani, Rossana;Trombetta, Carlo
2002-01-01
Abstract
The persistent mu¨llerian duct syndrome is a rare form of male intersex disorder in which a phenotypically normal male subject has fallopian tubes and a uterus along with testes and wolffian duct derivatives. This internal male pseudohermaphroditism is caused by a deficiency of mu¨llerian duct inhibitory factor.1 A genetic mutation of chromosome 19 seems to be implicated in this condition.2 Like other undescended testes these gonads are at increased risk of malignant transformation, and a variety of germ cell tumors have been reported in association with this syndrome.3 These patients often present with cryptorchidism or an inguinal hernia with fallopian tubes and a uterus. We report a case of bilateral synchronous germ cell tumor in a patient with the persistent mu¨llerian duct syndrome.Pubblicazioni consigliate
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