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Case Reports
. 2022 Feb 21:42:102031.
doi: 10.1016/j.eucr.2022.102031. eCollection 2022 May.

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

Abdullah B Alanazi  1 , Afrah Aldhowayan  2 , Mousa M Almuhanna  3 , Abdullah M Alghamdi  1
Affiliations
Case Reports

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

Abdullah B Alanazi et al. Urol Case Rep. .

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.

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Conflict of interest statement

No conflict of interest

Figures

Fig. 1
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
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
Fig. 3
Rudimentary uterus, and the left testicular mass.
See this image and copyright information in PMC

References

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    1. Hsi-Chin W. Persistent Müllerian duct syndrome with seminoma. Am J Roentgenol. 2000;174(1) (AJR). Ajronline.org. - PubMed
    1. Nilson O. Hernia uterus inguinalis beim manne. Acta Chir Scand. 1939;83:231–239.
    1. Al-Asmar A., Abu-Qamar A., Al-Saidah N., Al-Kaabneh A., Al-Qaralleh A., Al-Hiari A. Persistant Mullerian duct syndrome with intra-abdominal seminoma. Urol Case Rep. 2020 Jul 14;33:101342. - PMC - PubMed
    1. Ahadi M., Soleimantabar H., Javanmard B., Zahedifard S. A rare case of persistent Mullerian duct syndrome and review of literature. Int J Cancer Manag. 2020;13(3)

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