Case Reports in Clinical Practice 2017. 2(4):94-97.

Bilateral Incidental Gonadoblastoma in a Woman with Swyer Syndrome Presenting with Primary Amenorrhea
Shahram Shabaninia, Seyed Reza Yahyazadeh, Amin Azadfar, Mahrouz Amirheydari


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.



Amenorrhea; Swyer syndrome; Gonadoblastoma; Gonadal dysgenesis

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