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Mayer-Rokitansky-Küster-Hauser Syndrome With Bilateral Ovarian uterine agenesis|Sertoli cell|ovarian tumor|ovarian neoplasia|müllerian agenesis Tumors: Review of the Literature and Report of a Rare Case

Juusela, Alexander L., MD, MPH*; Naghi, Ilana, MD*; Thani, Suresh, MD, FACOG

Female Pelvic Medicine & Reconstructive Surgery: September/October 2018 - Volume 24 - Issue 5 - p e32–e34
doi: 10.1097/SPV.0000000000000483
Case Reports

Background Patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome are infertile secondary to hypoplasia or complete agenesis of the uterus, yet they remain at risk of primary neoplasms of the ovaries because embryologically the uterus and ovaries develop via separate mechanisms.

Case A 72-year-old nulliparous woman with a history of primary amenorrhea underwent an exploratory laparotomy for a suspected uterine fibroid. In addition to the pelvic mass, the patient was found to have findings consistent with MRKH syndrome. Postoperative pathological examination demonstrated bilateral ovarian uterine agenesis|Sertoli cell|ovarian tumor|ovarian neoplasia|müllerian agenesis tumors.

Conclusions The case presented is unique in that 2 rare pathologies, bilateral uterine agenesis|Sertoli cell|ovarian tumor|ovarian neoplasia|müllerian agenesis tumors of the ovary and MRKH syndrome, developed concomitantly in the same patient.

From the *Obstetrics and Gynecology Department, Newark Beth Israel Medical Center, Newark, NJ;

Private Obstetrics and Gynecology Practice, Irvington, NJ.

Correspondence: Alexander L. Juusela, MD, MPH, Obstetrics and Gynecology Department, Newark Beth Israel Medical Center, 201 Lyons Ave, Newark, NJ 07112. E-mail: Alex.l.juusela@gmail.com.

The authors have declared they have no conflicts of interest.

The Newark Beth Israel Medical Center institutional review board/ethics committee ruled that approval was not required for this case report.

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