Recent advances in the pathology and classification of gonadal neoplasms composed of germ cells and sex cord derivatives.

In recent years, our understanding of neoplasms composed of germ cells and sex cord derivatives has increased. In this review, advances in the classification and pathology of ovarian germ cell-sex cord-stromal tumors are discussed. Only 2 neoplasms, each with a distinctive pathogenesis and clinicopathologic features, are included in this category. Gonadoblastoma is a tumor that usually occurs in the dysgenetic gonads of intersex patients that have a Y chromosome, whereas mixed germ cell-sex cord-stromal tumor arises in normal gonads in patients without sex chromosomal abnormalities. Ovarian mixed germ cell-sex cord-stromal tumors differ from their testicular counterparts in their histological appearance, immunohistochemical staining reactions, and biological behavior probably because the latter show a greater degree of maturity of their germ cell component. The introduction of cisplatin-based chemotherapy and the application of tumor markers have dramatically improved the clinical outlook for those patients who develop secondary malignant germ cell neoplasms.