Three-Dimensional Computed Tomography of Pelvic Masses in Mayer-Rokitansky-Kuster-Hauser Syndrome

BACKGROUND: Mayer-Rokitansky-Kuster-Hauser syndrome is a rare congenital anomaly characterized by congenital aplasia or hypoplasia of the uterus and vagina. We report a case of Mayer-Rokitansky-Kuster-Hauser syndrome with multiple large pelvic masses diagnosed by three-dimensional computed tomography (CT) angiography. CASE: A 40-year-old woman with Mayer-Rokitansky-Kuster-Hauser syndrome presented with an abdominal mass that had grown for 3 months. Magnetic resonance imaging (MRI) confirmed several solid masses, and normal bilateral ovaries were detected; three-dimensional CT revealed that these tumors were supplied from the right ovarian and uterine arteries, suggesting that they arose from the uterus. Accordingly, leiomyoma was suspected. Laparoscopic surgery was contraindicated, and the patient therefore underwent laparotomy. The masses were resected with the bilateral rudimentary uteri and fallopian tubes, and pathologic evaluation confirmed leiomyoma. CONCLUSIONS: Combined MRI and three-dimensional CT angiography can accurately evaluate the origin and anatomic properties of leiomyomas in patients with Mayer-Rokitansky-Kuster-Hauser syndrome.