1104 Pediatric Adrenocortical Tumors. Twenty Five Years Experience in a University Hospital in Tehran, Iran

Abstract

Adrenocortical tumors are rare childhood and adolescent neoplasms. More than 95% of these tumors are functional and present with virilization, Cushing's syndrome, hypertension or hyperestrogenism. During 25 years of experience in Taleghani hospital, Tehran, Iran, we could find 7 cases of such tumors. Patients were 2 males and 5 females, aged 2-16 years. Five patients presented with virilization, one with a mixed picture of acute psychosis, cushing's syndrome and feminization and one with Cushing's syndrome. Duration of the disease was between 4 months to 5 years and 3 patients were erroneously diagnosed and treated as congenital adrenal hyperplasia. Diagnosis was confirmed with basal hormonal evaluation, suppression tests and appropriate imaging. All patients underwent open surgery. During surgery a blood sample was drawn from the adrenal vein of the affected side and its steroid contents were compared with the data obtained from the peripheral blood during and after surgery. Adrenal vein evaluation revealed that cortisol, testosterone and other hormones were directly secreted from the tumor and peripheral conversion had a limited contributory effect on plasma levels. The tumors weighed between 36 to 103 grams and while in cases #1&3 pleomorphic atypical nuclei with capsular and vascular invasion were reported, the patients had no signs or symptoms of relapse so far. Duration of follow up is 4 to 16 years and no case of relapse has been seen. Conclusion: Pediatric adrenocortical tumors are multi - hormone secreting neoplasms and have a more favorable prognosis in comparison to adults.