A Rare Case of Persistent Mullerian Duct Syndrome Vivekanand N1,*, Sudha Ayyagari2,**, Rahman SA3,*** 1MD, Associate Professor, Department of Pathology, Rajiv Gandhi Institute of Medical Sciences, Adilabad, Andhra Pradesh, India 2MD Consultant, Pathologist, Yashoda Hospitals, Hyderabad, Andhra Pradesh 3MD Consultant, Pathologist, Yashoda Hospitals Correspondence Author Email: *drnvivek2010@gmail.com
**ayyagarisudha@yahoo.com
***sarahman82@gmail.com
Online published on 15 May, 2014. 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. Top Keywords Cryptorchidism, Mullerian inhibiting factor, Persistent Mullerain duct syndrome, Uterusautosomal dominant, Malignant transformation. Top |