Abstract

Various types of gonadal neoplasia are encountered with unusual frequency in several forms of congenitally abnormal gonads associated with somatosexual maldevelopment. But the incidence of such an association is probably greater than it would seem to be from a review of the available metrical literature. This discrepancy is probably due to followings : 1) In some patients, the genital anomaly was obvious, but the "hidden" neoplasm was missed because physical examination, surgery, or autopsy was not performed. 2) In other patients, though a gonadal neoplasm was found, the sexual ambiguity was overlooked. 3) In still other instances, a gonadal tut was encountered in individuals whose external genitals were consistent with the attributed sex but whose genetic sex may have been contrary Nowadays, a simply method chromosomal analysis enhanced our understanding of this biological phenomenon helped to explain the various morphological aberrations seen in them. 4) Finally, there mayhave been a number of recognized intersexes with gonadal neoplasms who were not reported, because of the bizarre and poorly understood pathological findings. True hermaphroditism is the most uncommon varient of intersexuality in the human being and associated with malignant germ cell tumors of gonads are very rare. Most of them are dysgerminoma, gonadoblastoma and only few cases are endodermal sinus tumor. We describe in this paper an unusual case of true hermaphrodite with 46, XY chromosomal pattern and in whom, on exploration, we defected a huge gonadal mass on right side and an ovotestis on left side.