Congenital adrenal hyperplasia with an ovarian adrenal rest tumor: a case report

Abstract

Adrenal rest tumors (ART) are extra-adrenal findings of adrenal tissue. Testicular adrenal rest tumors (TART)are widespread in males with congenital adrenal hyperplasia (CAH). Ovarian adrenal rest tumours (OART),which form in females with CAH, are less commonly seen. Reports in literature of OART are extemely rare.The case is here presented of a 43-year-old female who was diagnosed at the age of 15 years with CAH andunderwent bilateral adrenalectomy at the age of 37. On presentation at our clinic, 4×2 cm mass was determinedon the right adnexa in the advanced tests and a right-side salpingo-oopherectomy was performed. The pathologyexamination reported heterotopic adrenal cortical tissue. Routine ovarian imaging in females with CAH is notindicated. Although OART are rarely seen, they should be kept in mind and these patients should be evaluatedradiologically together with laboratory tests.