Dysgerminoma of the ovary in association with XY gonadal dysgenesis

A case is reported of an adolescent presenting with primary amenorrhea. On pelvic examination a large unilateral ovarian tumor was palpated. Microscopic examination of the tumor removed at laparotomy revealed a pure dysgerminoma. Further gynecological-endocrinological investigations and chromosome analysis showed an XY gonadal dysgenesis. A review of current approaches to diagnosis, prognosis and especially to the controversial therapeutic modalities is presented.