A Rare Presentation of Germ Cell Neoplasia: Persistant Müllerian Duct Syndrome

Persistent Müllerian duct syndrome (PMDS) is defined as the presence of Müllerian remnants in a phenotypically and karyotypically male (1). PMDS is caused either by the lack of anti-Müllerian hormone, receptor insensitivity or a defect in the timing of the release of Müllerian inhibiting factor (MIF). PMDS is regarded as a form of pseudohermaphroditism due to presence of uterus and is shown to be inherited autosomal recessively (2). Although it is predominantly seen in pediatric age group presenting as undescended testis with or without inguinal hernia, it might be presented as an inguinal or intraabdominal mass in adulthood (3). Herein we report 2 cases of PMDS with review of the literature. Case Presentations