Steroid and gonadotropin secretion in a patient with a 30-year history of virilization due to lipoid-cell ovarian tumor

A 36-year-old woman with a 30-year history of undiagnosed virilizing lipoid-cell ovarian tumor is described. The tumor was localized by the NP-59 scan. Data of extensive investigations of steroid and gonadotropin secretion are provided. The preoperative dynamics of serum dehydroepiandrosterone sulfate and its decrease after the removal of the tumor suggested that dehydroepiandrosterone sulfate was being secreted by the ovarian tumor. Ovarian-peripheral venous gradients for plasma Δ5 and Δ4 steroids were correlated with the in vitro measurements of various steroidogenic enzymatic activities in the tumor tissue. The proposed metabolic pathway of the tumor is as follows: Pregnenolone → 17-hydroxypregnenolone → dehydroepiandrosterone → androstenedione → testosterone → estradiol. Low basal gonadotropin levels with no discernible pulsatility and no response to gonadotrop in-releasing hormone were observed preoperatively, and a gradual normalization in both parameters was observed during the six months after removal of the tumor.