Hysterectomy in a Male! Rare Stigma of a Case of Persistent Mullerian Duct Syndrome

Excerpt

Disorders of sex differentiation (DSD) are very important issues in clinical practice, particularly because of their rarity and difficulty pertaining to their diagnosis and management. Persistent Mullerian duct syndrome (PMDS) is a form of disorders of sex differentiation (DSD) caused by a defect in the Mullerian inhibiting substance (MIS) system. Patients are genotypically as well as phenotypically male but with the presence of Mullerian duct derivatives [1]. This entity is often misdiagnosed due to a lack of familiarity with the condition and requires a high index of suspicion on the operating surgeon’s part for its diagnosis. We are reporting such a rare case of persistent Mullerian duct syndrome where late presentation in part of patient resulted in development of testicular malignancy. …