A Rare Case of Persistent Mullerian Duct Syndrome
Date
2014-02
Authors
Journal Title
Journal ISSN
Volume Title
Publisher
Association of Medical Dental and Allied Sciences
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.
Description
Keywords
Cryptorchidism, Mullerian inhibiting factor, Persistent Mullerain duct syndrome, Uterusautosomal dominant, Malignant transformation
Citation
N Vivekanand, Ayyagari Sudha, Rahman SA. A Rare Case of Persistent Mullerian Duct Syndrome. Indian Journal of Mednodent and Allied Sciences. 2014 Feb; 2(1): 93-96