Steroid cell tumor of the ovary as a rare cause of virilization

Virilization is associated with either ovarian causes, including polycystic ovary syndrome, hyperthecosis, and ovarian tumor, or with adrenal causes, including tumors and congenital adrenal hyperplasia. In establishing the diagnosis, levels of dehydroepiandresterone sulfate, testosterone, and 17 alpha-hydroxyprogesterone (17-OHP), with their response to dexamethasone treatment, should be assessed; and, where indicated, computerized tomography, ultrasound, and selective venous catheterization should be undertaken.

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 had been treated with glucocorticoids, with no regression in virilization, 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 of corticotropin, led us to investigate an androgen-secreting tumor of ovarian origin. When ultrasonography and computerized tomography of the ovaries and adrenal glands displayed no abnormality, selective venous catheterization was performed, revealing an abnormal ovarian-peripheral gradient for testosterone, 17-OHP, estradiol, and androstenedione in the right ovarian vein. On exploratory laparotomy, a neoplasm adjacent 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 tumor removal.

Selective ovarian and adrenal venous catheterization for hormone assays is an efficient method of identification and localization of an androgen source in virilizing syndromes when noninvasive methods fail.