Mixed Germ Cell-Sex Cord-Stromal Tumor

Comments:
Mixed germ cell-sex cord-stromal tumor, unclassified, is an extremely rare ovarian tumor that shares some features with gonadoblastoma. However, it is found in phenotypic normal females with 46,XX karyotype in contrast to gonadoblastomas that develop in dysgenetic gonads in individuals with abnormal karyotypes. Just like gonadoblastomas, this tumor is capable of giving rise to invasive germ cell tumors such as dysgerminomas.

Most patients are genotypic and phenotypic normal young females. Some cases are associated with isosexual pseudoprecocity. Grossly, they are unilateral, solid, large tumors with yellow-tan cut surface.

This low magnification view shows admixture of dysgerminoma-like primitive germ cells and sex cord cells arranged in nests, separated by fibrous stroma. Scattered foci of calcification are also seen.