Malignant adrenal tumors.

Adrenal tumors, apart from neuroblastoma, are relatively rare in infancy and childhood. Most adrenal lesions are benign, and both benign and malignant tumors may be hormonally active thus, making accurate preoperative diagnosis difficult. The two main malignant tumors are adrenocortical carcinoma and pheochromocytoma. In both tumors, it may be difficult to determine benign from malignant and the biologic behavior and degree of invasion may portend a more malignant course. Surgical excision is the primary therapy for both tumors, including excision of metastatic and recurrent tumor. An open procedure should be considered for invasive adrenocortical carcinoma and in pheochromocytomas in which preoperative imaging demonstrates metastatic nodal disease. A laparoscopic approach is preferred for lesions in which preoperative imaging demonstrates a localized lesion. Chemotherapy, although without proven efficacy, is utilized in some children with metastatic or unresectable disease.