Ovarian and peripheral venous steroids in XY gonadal dysgenesis and gonadoblastoma

Determinations of multiple steroids were made on ovarian and peripheral blood in a 46,XY patient with bilateral gonadoblastoma. The right gonadoblastoma had undergone complete calcific ablation. The principal viable cellular elements in the left gonadoblastoma were Leydig cells. Except for a borderline increase over peripheral levels of estradiol, the degenerated right tumor mass showed no evidence of endocrine activity. Venous blood emanating from the left cellular gonadoblastoma exhibited significant elevations of testosterone, progesterone, and estradiol as compared to peripheral blood. Viable sex cord elements were present in the left tumor mass but did not exhibit sufficient steroid activity to have demonstrable clinical manifestations.