Reviewing recherche presentations of persistent Mullerian duct syndrome: case reports

Abstract

Abstract Background Persistent Mullerian Duct Syndrome (PMDS) is a type of pseudo-hermaphroditism occurring in males internally due to failure in the Anti Mullerian Hormone (AMH)-dependent sex differentiation pathway. Due to absent Anti Mullerian Hormone (AMH), the adult male has both the Wolffian and Mullerian duct derivatives. Considering the limited information available in the existing literature, malignant transformation in PMDS has rarely been reported. Case Presentation In this article we present two cases of PMDS which have complicated in to malignancy with review of literature and histopathological correlation. Both of these patients presented with lower abdominal pain, for which computed tomography of abdomen and pelvis was performed following ultrasound of abdomen and pelvis Both the Wolffian and Mullerian duct derivates were visualized on the above mentioned scans in these genetically confirmed males (46 XY). Conclusion Familiarity with PMDS is necessary to diagnose the condition. Hence, in this article, we report recherche presentations of Persistent Mullerian Duct Syndrome which had complicated into malignancies.

Keywords

• Persistent Mullerian duct syndrome
• Mullerian duct derivatives
• Anti Mullerian hormone
• Cryptorchidism
• Endodermal sinus tumour