Gonadoblastoma Occurring in a Patient with Familial Gonadal Dysgenesis

Recent morphologic and histogenetic studies provide a basis for the classification of testicular and ovarian tumors of germinal, mesenchymal, and sex cord origin. That gonadal tumors in the male and female are identical and homologous' 44 is attributed to totipotentiality of the cellular components that contribute to the formation of the gonad. Seminoma, embryonal carcinoma, teratoma, and choriocarcinoma represent examples of germinal derivatives, whereas tumors of Leydig and theca cells and those of granulosa and Sertoli cells represent, respectively, mesenchymal and sex cord derivatives. Testicular tumors of multiple cell type usually arise from the germinal epithelium. Participation of sex cord and mesenchymal elements in the same tumor is more generally found in the ovary. The presence of all 3 embryologic forerunners in 1 tumor, which is least frequently encountered, was first reported by Scully in 1953 under the name gonadoblastoma. This paper deals with the histologic and endocrine aspects of a case of gonadoblastoma and with a review of cases in the literature in which the morphologic or clinical findings, or both, were similar.