Laparoscopic management of ovarian cysts in peripheral precocious puberty of McCune-Albright syndrome.
Ovarian cysts are common in peripheral precocious puberty in McCune-Albright syndrome (MAS). The clinical course of these cysts is unpredictable due to episodes of hyperestrogenism typical of MAS ovarian hyperfunction. In persistent and recurrent large ovarian cysts with sustained estrogen hypersecretion and relevant clinical disturbances (increased linear growth and bone age maturation, vaginal bleeding and psychological disturbances) treatment is mandatory. Experimental courses of estrogen-blocking drugs may have insufficient or nil therapeutic effects. In these cases and when molecular analysis is required to obtain MAS diagnosis as in isolated peripheral precocious puberty, surgery is the option. Laparoscopy minimizes surgical aggression and facilitates obtaining tissue samples for molecular analysis, and sometimes relieves hyperestrogenism with the excision of hyperactive ovarian areas. It can be conducted with trans-umbilical laparoscopic ovarian cystectomy (TULOC) before 3 years of age and with traditional techniques afterwards.
Gesmundo
Roberto
R
B Division of Pediatric Surgery, University of Turin, Regina Margherita Children's Hospital, Turin, Italy.
Guanà
Riccardo
R
Valfrè
Laura
L
De Sanctis
Luisa
L
Matarazzo
Patrizia
P
Marzari
Daniela
D
Lala
Roberto
R
D002648:Child; D002675:Child, Preschool; D002864:Chromogranins; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications* / Q000235:genetics / Q000601:surgery; D019205:GTP-Binding Protein alpha Subunits, Gs / Q000235:genetics; D006801:Humans; D007223:Infant; D010535:Laparoscopy / Q000379:methods*; D009030:Mosaicism; D009154:Mutation; D010048:Ovarian Cysts / Q000209:etiology / Q000235:genetics / Q000601:surgery*; D011629:Puberty, Precocious / Q000150:complications / Q000235:genetics / Q000601:surgery*
D002864:Chromogranins; C406732:GNAS protein, human; D019205:GTP-Binding Protein alpha Subunits, Gs