A Case of 21-hydroxylase Deficiency with Massive Bilateral Adrenal Masses

Abstract

Background: Congenital adrenal hyperplasia (CAH) can lead to bilateral adrenal tumors. Excess adrenocorticotropic hormone is thought to play a role in the development of adrenal nodules. Here we present a patient with a simple virilizing form of 21-hydroxylase deficiency, a married man, bilateral adrenal tumors, and 46-XX chromosomes.Case report: 39-year-old man suffered from abdominal pain and tension. Abdominal tomography showed macronodular hyperplasia in both adrenal glands, with the largest nodule reaching 4.2 cm on the left side. The patient's old records showed that CAH had been diagnosed at the age of 11 years, but the patient was not taking any medication. The patient was treated with glucocorticoid. Despite irregular use of the treatment, the size of the nodules remained stable for 3 years, and then a significant reduction in nodule size was observed. Conclusion: In patients with bilateral adrenal masses and incidentaloma, CAH should be considered to avoid unnecessary surgery or biopsy. A 17-hydroxyprogesterone test in a suspicious patient is a useful tool for diagnosis.