Dysgerminoma in a 10-Year Old with 45X/46XY Turner Syndrome Mosaicism |
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Authors: | Tazim Dowlut-McElroy Denise A. Vilchez Eugenio M. Taboada Julie L. Strickland |
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Affiliation: | 1. Department of Surgery, Children''s Mercy Hospitals, Kansas City, MO;2. OB/GYN Specialists of the Palm Beaches, Wellington, FL;3. Department of Pathology and Laboratory Medicine, Children''s Mercy Hospitals, Kansas City, MO |
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Abstract: | BackgroundTurner 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.CaseA 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 ConclusionGonadoblastoma 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. |
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Keywords: | Turner syndrome Mosaic Gonadoblastoma Dysgerminoma |
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