A unique case of aggressive uterine cancer in a 45-year-old man with persistent Müllerian duct syndrome.

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.