Bilateral gonadoblastoma in a 17-year-old patient with 46XY pure gonadal dysgenesis (Swyer syndrome)

Gonadoblastomas are benign tumors composed of germ cells and sex cords derivatives resembling immature granulosa and Sertoli cells. They occur almost entirely in patients with gonadal dysgenesis and in those carrying the Y chromosome. The authors present a case of a 17-year-old patient admitted because of primary amenorrhea and delayed puberty diagnosed, and operated because of bilateral gonadoblastoma.

Gonadoblastoma; Gonadal dysgenesis; Swyer syndrome

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