Tumors of the adrenal gland in childhood. Report of 38 patients

During the past 17 years at the Department für Pediatric Surgery, University of Heidelberg, 38 children aged 1.6 to 14 years were adrenalectomized (unilateral 3.3, bilateral 5). Individual diagnoses were: neuroblastoma 23; pheochromocytoma 5; adrenocortical carcinoma 8; adrenocortical adenoma 4; bilateral nodular hyperplasia 2 cases. Patients with histologically benign lesions are alive and without recurrence more than 5 years after surgery, except one patient who developed Nelson's tumor after bilateral adrenalectomy for Cushing's disease. Of the patients with malignant adrenal tumors 21 died within 18 months after therapy was started, a 7 years old girl with an adrenal carcinoma died after a period of 3.6 years of combined treatment. In most cases of adrenocortical tumors virilization was the prominent feature.