Simultaneous Adrenal and Cervical Pheochromocytomas in Childhood

Abstract

Pheochromocytoma is an uncommon tumor in childhood. The simultaneous occurrence of adrenal and cervical pheochromocytomas is a rare phenomenon; to our knowledge, this combination has been reported in the literature only once. Cervical pheochromocytomas are more accurately termed "aorticosympathetic paragangliomas." In children, 50% of pheochromocytomas are bilateral, multiple, or extra-adrenal. An increased familial incidence in the form of simple mendelian dominance is also noted in the pediatric age group. Bolus nephrotomography is extremely effective in identifying adrenal pheochromocytomas in children. To prepare the patient for surgery, and alpha-adrenergic blocking agent is administered from 7 to 10 days before operation and a beta-blocking agent is administered 3 days before. A transabdominal approach is essential because of the frequent extra-adrenal sites and multicentricity of the tumor in children.