Huge Leiomyomas Arising from Bilateral Uterine Remnants in a Mayer–Rokitansky-Küster-Hauser Syndrome Patient with Coexisting Myotonic Dystrophy Type 1: A Case Report and Literature Review

Abstract

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a rare congenital anomaly of the genital tract. Since the secretion of sex hormones from the ovaries is preserved, leiomyomas and adenomyomas, which are estrogen-dependent diseases, may develop from the uterine remnant. In contrast, patients with myotonic dystrophy type 1 (DM1), the most common dystrophy in adults, are considered to be at high risk for benign tumors of the female reproductive system, such as uterine leiomyomas and ovarian cysts. A rare case of huge leiomyomas arising from bilateral uterine remnants in a woman with MRKHS with coexisting DM1 is presented. Her chief complaint was abdominal distension. On pelvic magnetic resonance imaging (MRI), two solid pelvic masses showing low signal intensity on T2-weighted imaging were seen. Both the uterine corpus and cervix were unclear, but bilateral ovaries were observed normally on MRI. Two uterine leiomyoma-like masses connected by a band of fibrous tissue were found by laparotomy. As with the MRI findings, the uterine cervix and vagina could not be detected macroscopically. Normal bilateral adnexa and round ligaments were identified. All of her symptoms improved after hysterectomy.