Mayer-rokitansky-kuster-hauser syndrome with presacral schwannoma presenting as a pelvic mass: A literature review and case report

A 32-year-old woman was admitted to our gynecology outpatient clinic with primary amenorrhea, a pelvic mass, and pain. Sonographic examination and magnetic resonance imaging revealed an approximately 124×103 mm heterogeneous mass. Moreover, laparotomy revealed fibrotic uterine bands with normal ovaries, tubes, and a solid retroperitoneal lesion. On the second postoperative day, the mass was removed, and the patient was discharged with full recovery. Microscopic examination of the pelvic mass confirmed the diagnosis of schwannoma. To the best of our knowledge, this is the first report on the co-occurrence of Mayer-Rokitansky-Küster-Hauser syndrome and schwannoma, without the presence of any other pathology.