Polycystic ovary and gonadoblastoma in Turner's syndrome
Maggio, M.C.; Liotta, A.; De Grazia, E.; Cimador, M.; Di Pace, R.; Corsello, G.
Minerva Pediatrica 59(4): 397-401
2007
ISSN/ISBN: 0026-4946 PMID: 17947845 Document Number: 611048
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.