Androgen-producing tumors of the adrenal and the ovary are extremely rare, however, their potential malignancy and the androgen effects can be detrimental to children and adolescents. Adrenal adenomas are usually small, whereas adrenal carcinomas are generally large and very aggressive, requiring prompt intervention. Adrenal tumors mostly secrete Δ5 androgens, although at least 12 cases of testosterone-producing adrenal tumors have been reported. One-third of functioning adrenal tumors also have increased secretion of corticosteroids. Primary androgen-producing ovarian tumors are classified under several different histological groups. Incidence of malignancy in these tumors is approximately 5%. Tumors metastatic to the ovary may stimulate the adjacent ovarian stroma and cause virilization. These must be differentiated from primary ovarian tumors because the prognosis is completely different. Ovarian tumors prefer Δ4 androgens, although cases mainly producing Δ5 androgens have been reported. The dehydroepiandrosterone sulfate and testosterone in serum are the best screening parameters for these tumors, although accurate diagnosis requires computed tomography for adrenal tumors and pelvic exam combined with ultrasonography for ovarian tumors. Venous catheterization studies are rarely indicated. Adrenal carcinoma requires aggressive surgical treatment, whereas adrenal adenomas and primary ovarian tumors can be treated with conservative unilateral procedures.
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Obstetrics and Gynecology