Functioning adrenocortical tumors in children with studies on steroid contents of adrenal venous effluent

Adrenocortical tumors (ACT) are rare in children and few reports regarding this can be found in Iranian Medical literature. In this paper we present the clinical and laboratory data of 6 children with ACT who were referred either to the office of one of the authors (MSG), or to the Department of Endocrinology at Taleghani General Hospital and underwent operations there. In order to shed lighter on the secretory behavior of the tumors, a blood sample was drawn during surgery from the adrenal vein of the affected site and the hormonal values were compared with the constituents of samples drawn simultaneously from the peripheral vein. Patients were 2 boys and 4 girls. 5 patients presented with virilizing manifestations including hirsutism, acne, muscle growth, clitoromegaly or increased penile size. One patient presented with acute psychosis and massive gynecomastia. 3 patients had been diagnosed as cases of congenital adrenal hyperplasia (CAH) and were under treatment with corticosteroids. In 3 patients despite hypercortisolism having been proved, the patients didn’t have any symptoms or signs of Cushing’s syndrome. Serum testosterone and Dehydroepiandrosterone Sulfate (DHEA-S) were 276-695 ng/dl and 256-1790 μg/dl respectively in those who presented with virilization. In 3 patients with hypercortisolism, levels of 24-hour urinary free cortisol after dexamethasone were 31-154 μg. Hormonal evaluation of the adrenal vein revealed that testosterone, DHEA-S, estradiol, 17 OH progesterone and cortisol were directly released from the tumor and peripheral interconversion had a limited, if any role in the serum level of steroids. Ultrasound and CT Scan easily demonstrated tumors in the affected adrenal gland. Surgery was uneventful in all cases. The tumors weighed 36-103 gram. During the follow-up period, which was 3 months to 11 years (mean 5 years), no relapse was found. According to the results, ACT should be kept in mind in the differential diagnosis of all children and adolescents presenting with isosexual or heterosexual precocious puberty. Surgery is the treatment of choice and tumors weighing 100 gr or less have a favorable prognosis.