Title

Dysgerminoma in a 10-Year Old with 45X/46XY Turner Syndrome Mosaicism.

Document Type

Article

Publication Date

10-2019

Identifier

DOI: 10.1016/j.jpag.2019.06.008

Abstract

BACKGROUND: Turner syndrome is a genetic disorder resulting from the absence of or structural abnormality of one X chromosome. The presence of Y chromosome material in girls with Turner syndrome confers an increased risk of benign and malignant germ cell tumor and prophylactic bilateral gonadectomy is recommended.

CASE: A 10-year-old Turner mosaic syndrome (45X/46XY) patient underwent prophylactic gonadectomy after unremarkable preoperative pelvic imaging. Histopathology showed a streak right gonad, and left gonad with gonadoblastoma with limited degree of infiltrating germinoma.

SUMMARYAND CONCLUSION: Gonadoblastoma and dysgerminoma have been reported in girls with Turner mosaic who carry Y chromosome material. Prophylactic gonadectomy should be considered in these girls without delay.

Journal Title

Journal of pediatric and adolescent gynecology

Volume

32

Issue

5

First Page

555

Last Page

557

MeSH Keywords

Castration; Child; Dysgerminoma; Female; Gonadoblastoma; Humans; Ovarian Neoplasms; Turner Syndrome

Keywords

Dysgerminoma; Gonadoblastoma; Mosaic; Turner syndrome

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