[Development of dysgerminomas from gonadoblastomas (report of 2 cases with XY gonadal dysgenesis)]

Gonadoblastomas have a propensity to give rise to malignant germ cell neoplasms. This report analyzes the clinicopathologic findings in two phenotypic females with features of 46, XY pure gonadal dysgenesis, who developed dysgerminomas in gonadoblastomas. All stages in the evolution of the dysgerminomas from the germ cells in gonadoblastomas were observed. The pathogenesis of the XY gonadal dysgenesis is briefly discussed. Finally the strict recommendation is given to remove the streak gonads with and without tumors in such individuals.