Leiomyomas of the bilateral rudimentary uterus in a patient with Mayer-Rokitansky-K^|^uuml;ster- Hauser syndrome: A case report.

Objective: To report a case of leiomyomas on the rudimentary uterus in a patient with Mayer-Rokitansky-Küster-Hauser syndrome. Design: Case report. Setting: Department of Obstetrics and Gynecology, Nagasaki University School of Medicine. Patient: A 39-year-old woman with Mayer-Rokitansky-Küster-Hauser syndrome presented with lower abdominal pain and a pelvic mass.Intervention: Diagnostic and therapeutic laparoscopy. Main Outcome Measure: Histologic examination. Result: Laparoscopy revealed leiomyomas on the rudimentary uterus. Further histologic examination of a biopsy suggested tissues consistent with endometrioid appearance that clearly demonstrated expression of the estrogen and progesterone receptors when probed with monoclonal antibodies. However, this endometrium did not consist of the typical basal and functional layers, and the patient reported no response to hormonal withdrawal.Conclusion: Leiomyomas of the bilateral rudimentary uterus were diagnosed in this MRHS patient via laparoscopic surgery. Normal endometrium should have a functional layer and basal layer, which could not be established via histologic examination in this case. Furthermore, the basal layer demonstrated estrogen and progesterone receptors, but did not possess the ability to exfoliate, indicating that this endometrium is non-functional. These results suggest a relationship between the basal layer and the lack of normal endometrial function in the rudimentary uterus of this patient with Mayer-Rokitansky-Küster-Hauser syndrome.