A Rare Case of Persistent Mullerian Duct Syndrome

ABSTRACT

We report a rare case of Persistent Mullerain Duct Syndrome (PMDS) in a 16-year-old patient with bilateral undesceneded testes. On pathologic examination a uterus-like structure was seen which on histology showed endometrium and fallopian tube. Persistent Mullerian duct syndrome is a very rare form of male pseudo hermoaphroditism caused by lack of regression of Mullerian ducts in phenotypically and genotypically (46XY) male individuals. Mullerian duct derivatives include uterus, cervix, fallopian tubes and upper two-thirds of vagina. Mullerian ducts normally regress in male fetus at 8 weeks due to anti-Mullerian hormone (AMH), which binds to anti-Mullerian type 2 receptor causing disappearance of Mullerian ducts at 10 weeks of fetal age. Only about 200 cases have been described in literature so far.