OBJECTIVE: Persistent Müllerian Duct Syndrome (PMDS), one of the causes of male pseudohermaphroditism, is a rare syndrome characterized by the presence of internal female genitalia (uterus, fallopian, tubes, cervix and upper vagina) in otherwise phenotypically and normally virilized men. METHODS: We present the 4th documented case of uterine malignancy in a 45-year-old man with PMDS presenting with lower abdominal protuberance and hematuria.
RESULTS: Although testicular malignancies are common in undescended testis associated with PMDS, very few cases of müllerian duct malignancies have been reported.
CONCLUSIONS: Persistent mullerian duct syndrome can be associated with aggressive non-testicular malignancies, especially uterine cancer, in normally virilized males.