title: Müllerian remnant leiomyomas in women with Mayer-Rokitansky-Küster-Hauser syndrome.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.affiliation: From the Department of Obstetrics and Gynaecology University of the West Indies, and the Windward Medical Centre, Kingston, Jamaica.
authors.forename: Horace Melford
authors.initials: HM
authors.lastname: Fletcher
authors.forename: Karen
authors.initials: K
authors.lastname: Campbell-Simpson
authors.forename: Dwight
authors.initials: D
authors.lastname: Walcott
authors.forename: John
authors.initials: J
authors.lastname: Harriott
mesh_terms: D058489:46, XX Disorders of Sex Development / Q000150:complications*; D000015:Abnormalities, Multiple; D000291:Adnexal Diseases / Q000209:etiology* / Q000601:surgery; D000328:Adult; D000013:Congenital Abnormalities; D005260:Female; D006801:Humans; D007668:Kidney / Q000002:abnormalities; D007889:Leiomyoma / Q000150:complications* / Q000601:surgery; D009095:Mullerian Ducts / Q000473:pathology* / Q000002:abnormalities; D019170:Somites / Q000002:abnormalities; D013131:Spine / Q000002:abnormalities; D014102:Torsion Abnormality / Q000209:etiology* / Q000601:surgery; D014430:Twins, Monozygotic; D014599:Uterus / Q000002:abnormalities; D014621:Vagina / Q000002:abnormalities