Malignant teratoma arising in a dysgenetic gonad

A case of malignant teratoma arising within a dysgenetic gonad in a 21- year-old phenotypic female with a 46 XY karyotype is presented. Admixtures of dysgerminoma, yolk sac tumor in close juxtaposition to embryoid bodies and elements of choriocarcinoma were also present. The contralateral gonad was an unidentifiable fibrovascular streak. Neither gonadoblastoma nor coarse calcifications (such as commonly found in gonadoblastoma) could be identified. We believe that the present case arose de novo in a dysgenetic gonad and, uncharacteristically, was not associated with a gonadoblastoma.