Adrenal tumors

Abstract

Neuroblastoma is by far the most common primary adrenal tumor in children, but other tumors are seen with some frequency and are treated differently. Adrenal tumors are a heterogeneous group and can be benign or malignant but for most surgery is the primary mode of therapy. Adrenocortical carcinomas are very rare in children, accounting for less than 0.2 % of all pediatric malignancies. A significant number of patients with adrenocortical tumors have a p53 mutation. Although adrenocortical carcinoma presents as an asymptomatic mass in about 10 % of cases, it frequently presents with evidence of hormonal stimulation. The absence of effective chemotherapy for adrenocortical carcinoma dictates aggressive attempts at resection. Although pheochromocytomas are seen in association with hereditary syndromes including MEN-2A, MEN-2B, and NF-1, most tumors are sporadic. Most pediatric patients with pheochromocytoma have signs and symptoms of sustained rather than paroxysmal hypertension. The primary treatment for pheochromocytoma is surgical resection.