Rare Case of Uterine Fibroids in Rudimentary Uterus of a Patient with Mayer-Rokitansky-Kuster-Hauser Syndrome: Minimally Invasive Robotic-Assisted Surgical Approach

Introduction: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a Mullerian congenital disorder.1 defined as agenesis or aplasia of the uterus and the upper vagina.2 We present a case of a 38-year-old female patient with primary amenorrhea who was already diagnosed with MRKH syndrome, referring pelvic pain and abdominal bulging for one year. On her abdominal physical exam, she had palpable abdominal tumors 2 cm below the umbilicus. Pelvic magnetic resonance imaging (MRI) showed two rudimentary horns with large nodules (10 cm and 7 cm) compatible with leiomyomas; however, both with hypersignal on T2, suggesting degeneration or atypia. MRI also showed left hematosalpinx, and the CA 125 level was increased (178—reference <35). Due to the atypical imaging and increased tumor marker, we opted for the surgical removal of the rudimentary uterus along with the pelvic masses and bilateral salpingo-oophorectomy. The pathology report confirmed the diagnosis of uterine leiomyomatosis and tubal endometriosis with no malignancy or atypia.