Persistent Mullerian duct syndrome (PMDS): Case report and review of literature

Abstract

Persistent Mullerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism (MPH) which characterized by the presence of uterus, fallopian tubes and upper part of vagina in an otherwise normally differentiated 46, XY male. The syndrome is caused by either an insufficient amount of antimüllerian hormone AMH or by insensitivity of the target organ to this factor, It is usually discovered in patient who assessed for infertility, cryptorchidism or during intra-abdominal surgery. Herein We report a rare presentation case of PMDS with intra-abdominal mass which came to be seminoma.

Keywords: Cryptorchidism; Germ cell tumor; PMDS.

Fig. 1

The left testis is being replaced by a large mass about 8.5 × 8.1 × 8.9 cm, The mass is inseparable from rudimentary uterus like structure at inferior aspect of side of the mass.

Fig. 2

Multiple paraaortic lymph nodes at the level of left renal helium, which forming a large mass measuring about 9.3 × 8 × 7.9 cm in transverse, anteroposterior and craniocaudal dimension respectively causing compression of left renal collecting system that causing moderate hydronephrosis despite the presence of the left double J stent.

Fig. 3

Rudimentary uterus, and the left testicular mass.