STEROID CELL TUMOR OF THE OVARY AS A RARE CAUSE OF VIRILIZATION

Background: Virilization is associated with either ovarian causes, inc luding polycystic ovary syndrome, hyperthecosis, and ovarian tumor, or with adrenal causes, including tumors and congenital adrenal hyperpla sia. In establishing the diagnosis, levels of dehydroepiandresterone s ulfate, testosterone, and 17 alpha-hydroxyprogesterone (17-OHP), with their response to dexamethasone treatment, should be assessed; and, wh ere indicated, computerized tomography, ultrasound, and selective veno us catheterization should be undertaken. Case Report and Results: A 21 -year-old woman presented with a 17-year history of early accelerated linear growth and virilization. During this time, a putative diagnosis of nonclassic congenital adrenal hyperplasia had been made, and she h ad been treated with glucocorticoids, with no regression in virilizati on, for 8 years. On presentation to our group, the failure of low- and high-dose dexamethasone suppression tests to decrease blood levels of testosterone and 17-OHP, combined with a relatively low blood level o f corticotropin, led us to investigate an androgen-secreting tumor of ovarian origin. When ultrasonography and computerized tomography of th e ovaries and adrenal glands displayed no abnormality, selective venou s catheterization was performed, revealing an abnormal ovarian-periphe ral gradient for testosterone, 17-OHP, estradiol, and androstenedione in the right ovarian vein. On exploratory laparotomy, a neoplasm adjac ent to the right ovary was resected and was found to be a steroid cell tumor of the ovary not otherwise specified, one of the rarest tumors causing virilization in children. Symptomatic resolution followed tumo r removal. Conclusion; Selective ovarian and adrenal venous catheteriz ation for hormone assays is an efficient method of identification and localization of an androgen source in virilizing syndromes when noninv asive methods fail.