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Treatment & Prognosis: Gonadoblastima is a benign tumor that does not metastasize. However, about 50% of cases develop invasive germ cell tumor (GCT) which determines the prognosis. The malignant component is a dysgerminoma in 80% of cases. Gonadoblastoma with dysgerminoma still has excellent prognosis. The outlook worsens if embryonal carcinoma or choriocarcinoma are found. When an invasive GCT is found in a gonadoblastoma, appropriate metastatic work-up and follow-up are indicated.

Due to the high risk of gonadoblastoma and an associated invasive GCT, patients with XY gonadal dysgenesis should under prophylactic bilateral gonadectomy in early childhood or after puberty. Virilizing symptoms regress after gonadectomy. Uterus may be left behind. Cyclic hormonal therapy is given to initiate menstrual periods.

Image 179 of 183