[Clinical features and outcomes of congenital adrenal hyperplasia with adenomatoid adrenal gland].

ABSTRACT

Objective: To analyze the clinical features and outcomes of congenital adrenal hyperplasia (CAH) with adenomatoid adrenal gland. Methods: Nineteen patients clinically confirmed as CAH with adenomatoid adrenal gland, from 2008 to 2015 in Deparment of Endocrinology of Chinese PLA General Hospital, were retrospectively analyzed, and the outcomes of the treatment were followed up. Results: Seventy-six patients were clinically confirmed as CAH, from 2008 to 2015 in PLA hospital. Nineteen of them have accompanied with adenomatoid adrenal gland. Of the 19 confirmed cases, 7 were male, and 12 were female. The mean age was (33.3±14.8) years old. Nineteen confirmed cases were consisted of 10 cases of 21-hydroxylase deficiency (OHD), 6 cases of 17α-OHD, and 3 cases of 11ß-OHD. Of the 19 cases, 5 cases presented with adrenal hyperplasia with left side adenomatoid solid lesion, 2 cases presented with adrenal hyperplasia with right side adenomatoid solid lesion, and 8 cases presented with double side adenomatoid solid lesion. The proportion of adrenal hyperplasia with unilateral cystic or calcified changes was 21.1%. Nine of the 19 patients underwent operation or fine needle biopsy. The pathology showed 7 adenomas (21-OHD/17α-OHD 5/2) and 2 myelolipomas (21-OHD/17α-OHD 1/1). Four patients were admitted into hospital because of the incidentaloma, while others because of the clinical symptoms. The average follow-up time was 31 months. CT images were rechecked among 7 patients. After the treatment, 1 patient's tumor shrunk, 4 cases had no recurrence, 1 case's tumor disappeared, and the other one case had no change. Symtoms of eleven followed-up patients relieved. Conclusions: The image of adrenal of CAH with adenomatoid adrenal gland is various. It may occur in each type of CAH presented as unilateral or bilateral, adenoma or myelolipoma.