BACKGROUND: Müllerian remnant leiomyomas occur in women with Mayer-Rokitansky-Küster Hauser syndrome in which leiomyomas arise in absence of a uterus. This is a rare condition and we present two unusual cases. CASES: In the first case, a woman with Mayer-Rokitansky-Küster-Hauser syndrome diagnosed was found to have a pelvic mass and was scheduled for laparotomy. A few days before the surgery, acute abdominal pain developed. At surgery she was found to have a twisted adnexum including a Müllerian remnant leiomyoma. This was untwisted successfully; the leiomyoma was excised and the ovary was saved. In the second case, one member of a pair of monozygotic twins was found to have leiomyomas at age 33 after having an absent uterus diagnosed at laparoscopy 8 years previously. She had successful removal of the leiomyoma at laparotomy. Her sister had normal development and had two children. CONCLUSION: Mayer-Rokitansky-Küster-Hauser syndrome leading to Müllerian remnant leiomyomas is uncommon but should be suspected in women in whom a pelvic mass develops after the confirmation of the Müllerian defect.
BACKGROUND: Müllerian remnant leiomyomas occur in women with Mayer-Rokitansky-Küster Hauser syndrome in which leiomyomas arise in absence of a uterus. This is a rare condition and we present two unusual cases. CASES: In the first case, a woman with Mayer-Rokitansky-Küster-Hauser syndrome diagnosed was found to have a pelvic mass and was scheduled for laparotomy. A few days before the surgery, acute abdominal pain developed. At surgery she was found to have a twisted adnexum including a Müllerian remnant leiomyoma. This was untwisted successfully; the leiomyoma was excised and the ovary was saved. In the second case, one member of a pair of monozygotic twins was found to have leiomyomas at age 33 after having an absent uterus diagnosed at laparoscopy 8 years previously. She had successful removal of the leiomyoma at laparotomy. Her sister had normal development and had two children. CONCLUSION: Mayer-Rokitansky-Küster-Hauser syndrome leading to Müllerian remnant leiomyomas is uncommon but should be suspected in women in whom a pelvic mass develops after the confirmation of the Müllerian defect.
Authors: Federico Romano; Stefania Carlucci; Guglielmo Stabile; Giuseppe Mirenda; Mariateresa Mirandola; Francesco Paolo Mangino; Andrea Romano; Giuseppe Ricci Journal: Int J Environ Res Public Health Date: 2021-05-31 Impact factor: 3.390