Pheochromocytoma in children

Abstract

The results of an examination, treatment, and postoperative follow-up of 520 patients with pheochromocytomas who were operated on at the Endocrinology Research Center, Russian Academy of Medical Sciences, in 1957 to 2002 have been analyzed. The patients’ mean age was 39.3±9.2 years; 50 (9.6%) patients aged less than 16 years. There were 213 (41.0%) males in the total group and 32 (62.0%) in the pediatric group. Bilateral adrenal lesion was detected in 68 (13.1%), including 16 (32%) of the 50 children aged less than 16 years. Extraadrenal tumors were ascertained in 36 (6.9%). The concomitance of pheochromocytoma with hereditary syndromes was observed in 36 (6.9%) cases. Two hundred and sixty patients were followed up for 4 to 25 years (mean 8.4+1.9). Recurrence ofpheochromocytoma was revealed in 49 (18.8%) patients; the genuine recurrence (a tumor at the site of primary surgery or metastasis) was found in 16 (6.15%) patients. The genuine recurrence of pheochromocytoma was seen in 6 (12.0%) of the 50 children; its main cause was organ-preserving surgery for multicenter adrenal lesion.