Bilateral Incidental Gonadoblastoma in a Woman with Swyer Syndrome Presenting with Primary Amenorrhea
Swyer syndrome is a very rare cause of primary amenorrhea. Affected individuals have an XY karyotype but their external and internal genitalia are of the female type. The gonads are usually replaced by fibrous streaks. Early diagnosis is vital because of the significant risk of germ cell tumor, and...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Tehran University of Medical Sciences
2018-05-01
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| Series: | Case Reports in Clinical Practice |
| Subjects: | |
| Online Access: | https://crcp.tums.ac.ir/index.php/crcp/article/view/135 |
| Summary: | Swyer syndrome is a very rare cause of primary amenorrhea. Affected individuals have an XY karyotype but their external and internal genitalia are of the female type. The gonads are usually replaced by fibrous streaks. Early diagnosis is vital because of the significant risk of germ cell tumor, and bilateral gonadectomy should be performed. Laparoscopy provides a minimally invasive approach for the management of these cases. These patients can have a normal sexual intercourse and they need hormone replacement therapy for development of breast and prevention of osteoporosis. They can conceive through oocyte donation and artificial reproductive techniques.
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| ISSN: | 2538-2683 2538-2691 |