Choriocarcinoma with teratoma arising from an intra-abdominal testis in patient with persistent Müllerian duct syndrome

Abstract

Persistent Müllerian duct syndrome (PMDS) is a rare form of internal male pseudohermaphroditism caused by failure of the fetal testis to produce Müllerian-inhibiting substance or failure of the tissues to respond to this hormone. Patients usually present in childhood with cryptorchidism or with an inguinal hernia. As with undescended testes, these gonads are at an increased risk of malignant transformation. We discuss a rare case of PMDS presenting with advanced metastatic choriocarcinoma with teratoma arising from an intra-abdominal testis after failed bilateral orchidopexy in childhood.