title: Restoration of ovulation after unilateral ovariectomy in a woman with McCune-Albright syndrome: a case report.
abstract: BACKGROUND
  McCune-Albright syndrome (MAS) is characterized by peripheral precocious puberty, café-au-lait spots, and polyostotic fibrous dysplasia. This syndrome is due to a post-zygotic mutation of the GNAS1 gene with mosaic distribution and unilateral predominance. Clinical manifestations depend on the tissues carrying the mutation. We describe the ovarian function before and after unilateral ovariectomy in a woman with MAS and bilateral distribution of the GNAS1 gene mutation.
  
  
  METHODS
  A 33-year-old patient, previously diagnosed as having MAS, presented irregular menstrual cycles (30-180 days) and monophasic temperature curves. Transvaginal ultrasound and blood tests were repeated at 3-day intervals over 3 months. Findings included a persistent quiescent left ovary, a persistent polycystic right ovary, constantly high estradiol-17beta (E2) levels, and very low FSH and LH levels. She also presented severe persistent pelvic pain. Because of unilateral ovarian activity, a unilateral right ovariectomy was performed as well as biopsy of the remaining left ovary. A GNAS1 gene mutation was identified in both ovaries. A regular monthly menstrual cycle was immediately restored. On day 3 of the menstrual cycle, E2 level was 30 pg/ml, FSH level was 7.5 mIU/ml, and LH level was 6.4 mIU/ml. On day 17, pelvic ultrasound showed one follicle of 25 mm in the left ovary. On day 21, the progesterone level was 13.1 ng/ml.
  
  
  CONCLUSIONS
  This is the first report of ovulation being restored following unilateral ovariectomy in an adult patient suffering from severe MAS with GNAS1 gene mutation identified in both ovaries.
authors.affiliation: Department of Obstetric Gynecology, University Hospital, Rennes, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Lavoué
authors.forename: Karine
authors.initials: K
authors.lastname: Morcel
authors.forename: Philippe
authors.initials: P
authors.lastname: Bouchard
authors.forename: Charles
authors.initials: C
authors.lastname: Sultan
authors.forename: Catherine
authors.initials: C
authors.lastname: Massart
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Grall
authors.forename: Serge
authors.initials: S
authors.lastname: Lumbroso
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Laurent
mesh_terms: D000328:Adult; D002864:Chromogranins; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000235:genetics / Q000503:physiopathology / Q000601:surgery*; D019205:GTP-Binding Protein alpha Subunits, Gs / Q000235:genetics; D006801:Humans; D009154:Mutation; D010052:Ovariectomy / Q000379:methods*; D010060:Ovulation / Q000502:physiology*
chemical_list: D002864:Chromogranins; C406732:GNAS protein, human; D019205:GTP-Binding Protein alpha Subunits, Gs