July 1990

Metastatic Pheochromocytoma Associated With Multiple Endocrine Neoplasia Syndrome Type II

Author Affiliations

From the Division of Surgical Oncology, Department of Surgery (Drs Chevinsky and Minton), and the Department of Medicine (Dr Falko), Ohio State University College of Medicine, Columbus.

Arch Surg. 1990;125(7):935-938. doi:10.1001/archsurg.1990.01410190133023

Pheochromocytoma is a rare adrenal medullary tumor of chromaffin cell origin that presents a syndrome of α- and β-adrenergic receptor stimulation due to secretion of epinephrine and norepinephrine. This tumor occurs sporadically in the population and is also associated with multiple endocrine neoplasia syndrome type II (MEN II). Although malignant pheochromocytoma is associated with sporadic tumors, those associated with familial MEN syndromes are rarely malignant. We report a case of a rare metastatic pheochromocytoma in a patient with MEN IIA. Surgical debulking, which has been shown to benefit many patients with metastatic neuroendocrine tumors, was attempted in this patient. Palliation, with symptom relief, was provided. The options for treatment of metastatic pheochromocytoma are discussed.

(Arch Surg. 1990;125:935-938)