title: Polycystic ovary and gonadoblastoma in Turner's syndrome.
abstract: Turner's syndrome (TS) is characterized by typical facial features, short stature, hypergonadotropic hypogonadism, streak gonads, infertility, hearth and kidney malformations. Typical karyotype is 45,X0; however, 6% of TS have mosaic patterns including Y chromosome or fragments of Y. This karyotype is a risk factor of developing a dysgerminoma in dysgenic gonads. Furthermore, rare cases of polycystic ovary are described in young-adult patients with TS. We describe the clinical case of a 12-year-old girl with TS treated with GH who showed a good response to treatment. She developed an ovary with histological polycystic pattern and a contralateral gonadoblastoma in the streak gonad. Laparoscopic gonadectomy was performed, with a good prognosis. Of remark is the opportunity to carry out gonadectomy in prepubertal age in girls with TS and Y chromosome material. This is a rare precocious case of polycystic ovary in TS, with different evolution in the two gonads with different histological differentiation.
authors.affiliation: Maternal Infantile Department, University of Palermo, Palermo, Italy. benfri@diseg.unipa.it
authors.forename: M C
authors.initials: MC
authors.lastname: Maggio
authors.forename: A
authors.initials: A
authors.lastname: Liotta
authors.forename: E
authors.initials: E
authors.lastname: De Grazia
authors.forename: M
authors.initials: M
authors.lastname: Cimador
authors.forename: R
authors.initials: R
authors.lastname: Di Pace
authors.forename: G
authors.initials: G
authors.lastname: Corsello
mesh_terms: D002648:Child; D005260:Female; D018238:Gonadoblastoma / Q000209:etiology* / Q000601:surgery; D006801:Humans; D010051:Ovarian Neoplasms / Q000209:etiology* / Q000601:surgery; D011085:Polycystic Ovary Syndrome / Q000209:etiology* / Q000601:surgery; D014424:Turner Syndrome / Q000150:complications*