title: McCune-Albright syndrome (MAS): early and extensive bone fibrous dysplasia involvement and "mistaken identity" oophorectomy.
abstract: BACKGROUND
  McCune-Albright syndrome (MAS) is a triad of gonadotropin-independent precocious puberty (GIPP), café-au-lait spots (CALS) and fibrous dysplasia (FD) of bone. The extent of the abnormalities is variable.
  
  
  RESULTS
  We report a 3 year old girl with CALS since infancy, FD diagnosed at age of 2.5 years, and at the age of 3 years vaginal bleeding. The ultrasound revealed a cystic mass of the ovary, surgical pathology found ovarian cyst. LHRH stimulation demonstrated GIPP (LH 9.8 mIU/ml and FSH 8.9 mIU/ml; normal LH 1.8-10, FSH 9-26 mIU/ml). Radiographs and bone scans demonstrated FD in multiple bones. Peripheral leucocytes and the ovary were negative for GNAS gene mutations. Treatment with Letrasole interrupted the pubertal development.
  
  
  CONCLUSIONS
  We conclude that the clinical signs of MAS are telling and that timely MAS diagnosis prevents unnecessary oophorectomy. A close follow up is recommended regarding development of endocrine disorders and spreading of FD.
authors.affiliation: Faculty of Medicine Skopje, 50 Divizija BB, 1000 Skopje, Macedonia. gucevz@gmail.com
authors.forename: Zoran
authors.initials: Z
authors.lastname: Gucev
authors.forename: Velibor
authors.initials: V
authors.lastname: Tasic
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Jancevska
authors.forename: Marina
authors.initials: M
authors.lastname: Krstevska-Konstantinova
authors.forename: Nada
authors.initials: N
authors.lastname: Pop-Jordanova
mesh_terms: D047072:Aromatase Inhibitors / Q000627:therapeutic use; D002675:Child, Preschool; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000175:diagnosis* / Q000188:drug therapy; D005640:Follicle Stimulating Hormone / Q000097:blood; D007987:Gonadotropin-Releasing Hormone / Q000097:blood; D006801:Humans; D000077289:Letrozole; D007986:Luteinizing Hormone / Q000097:blood; D009570:Nitriles / Q000627:therapeutic use; D010048:Ovarian Cysts / Q000000981:diagnostic imaging / Q000601:surgery; D010052:Ovariectomy; D011629:Puberty, Precocious / Q000175:diagnosis; D014230:Triazoles / Q000627:therapeutic use; D014463:Ultrasonography; D014592:Uterine Hemorrhage / Q000473:pathology
chemical_list: D047072:Aromatase Inhibitors; D009570:Nitriles; D014230:Triazoles; D007987:Gonadotropin-Releasing Hormone; D000077289:Letrozole; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone