Dysgerminoma of the ovary associated with true hermaphroditism

Abstract

A dysgerminoma of the left gonad exhibiting malignant features was diagnosed at laparotomy in a 33-year-old virginal phenotypic female who had a normal menstrual history. Pathologic examination of the contralateral (right) gonad revealed an ovotestis. The nonaffected residual portion of the tumor-bearing left gonad exhibited only ovarian stroma. The ovotestis was characterized by a nodule of testicular parenchyma composed of partly hyalinized seminiferous tubules without evident spermatogenesis. The ovotestis was contiguous to normal ovarian elements, including a cystic corpus luteum and primordial follicles. Proliferative endometrium was found lining the small uterus. The findings are indicative of true hermaphroditism. The occurrence of dysgerminoma and other tumors in association with various forms of gonadl dysgenesis is discussed. To the authors' knowledge, this is the first reported case of dysgerminoma occurring in a phenotypic female hermaphrodite.