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Minerva Pediatrica 2007 August;59(4):397-401

Copyright © 2007 EDIZIONI MINERVA MEDICA

language: English

Polycystic ovary and gonadoblastoma in Turner’s syndrome

Maggio M. C., Liotta A., De Grazia E., Cimador M., Di Pace R. M., Corsello G.

Maternal Infantile Department University of Palermo, Palermo, Italy


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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.

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