Persistent Mullerian Duct Syndrome- a Rare Case with Review of Literature

Abstract

Persistent Mullerian Duct Syndrome (PMDS) is a rare type of male pseudohermaphroditism. It is brought on by an insufficiency of anti-mullerian hormone (AMH) or a malfunction of its receptor (type II). Approximately 150 adult cases of PMDS have been documented overall, with the majority from the Middle East, USA and Europe. 44 publications (49 individuals) with 3 testicular tumors linked to PMDS were found using the Medline/Pubmed search; the majority (59%) of the patients presented with a big abdominal mass. In our case, the abdomen mass that was identified as seminoma testis in a 22-year-old male patient with PMDS. Our goal in presenting this case is to highlight how uncommon it is for PMDS patients to have a testicular tumor, and how the presence of an undescended testis raises the risk of testicular tumor development in PMDS patients. The clinicians should be aware of rare entity while dealing with the cryptorchidism with inguinal hernia and necessary management should be done to offer proper treatment.