Surgical management of pheochromocytoma in children

Abstract

A review is presented of seven children under the age of 18 yr who underwent surgical excision of pheochromocytomas. Twelve partial or total adrenalectomies were performed. Because of the high occurrence of bilateral tumors (70%), it is recommended that a biopsy be considered on the contralateral gland even though it appears normal at operation. Total adrenalectomy is recommended whenever a pheochromocytoma is present in the adrenal gland of a child since recurrence of the tumor in the residual gland after a subtotal resection is likely; the two recurrent tumors in this series occurred after subtotal adrenalectomy. Identical female twins had associated medullary thyroid carcinoma and pheochromocytoma (Sipple's syndrome). Two male siblings each had bilateral pheochromocytomas. Only one of the 12 tumors removed in this series was invasive. Aortography and selective venous catheterization were the most helpful diagnostic studies in establishing the diagnosis of pheochromocytoma. A review is presented of seven children under the age of 18 yr who underwent surgical excision of pheochromocytomas. Twelve partial or total adrenalectomies were performed. Because of the high occurrence of bilateral tumors (70%), it is recommended that a biopsy be considered on the contralateral gland even though it appears normal at operation. Total adrenalectomy is recommended whenever a pheochromocytoma is present in the adrenal gland of a child since recurrence of the tumor in the residual gland after a subtotal resection is likely; the two recurrent tumors in this series occurred after subtotal adrenalectomy. Identical female twins had associated medullary thyroid carcinoma and pheochromocytoma (Sipple's syndrome). Two male siblings each had bilateral pheochromocytomas. Only one of the 12 tumors removed in this series was invasive. Aortography and selective venous catheterization were the most helpful diagnostic studies in establishing the diagnosis of pheochromocytoma.