Ovarian adrenal rest tumor in a patient with salt-wasting form of congenital adrenal hyperplasia

Abstract

The article describes the clinical case of diagnosing and choosing the tactics for treating ovarian adrenal rest tumors (OART) in a patient with a salt-wasting form of congenital adrenal hyperplasia (CAH). There are only several clinical cases descriptions of OART in the world literature. At present, the tactics of observation and treatment of the ovarian adrenal rest tumors has not been developed. In each case, individual tactics are chosen. The main predisposing factor to the occurrence of OART is considered to be long periods of decompensation of CAH. ACTH stimulates the hyperplasia and hypertrophy of the residual adrenal tissue in the ovaries and secondary tumors develop. The instrumental methods that allow to diagnose OART include ultrasound, MRI of the pelvic organs, PET with 18FDG. The tactics of treating OART, in contrast to the testicular adrenal rest tumors in men (TART), according to world literature are mainly surgical. Organ-preserving operations were performed, as well as the removal of tumors along with the ovary. Cases of a combination of adenocarcinoma and CAH are also described. Each new case of OART is unique and with the accumulation of international experience, a consensus will be developed.