Bilateral testicular carcinoma in patient with the persistent müllerian duct syndrome

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.