Metastatic pheochromocytoma associated with multiple endocrine neoplasia syndrome type II
A. H. Chevinsky, J. P. Minton and J. M. Falko
Department of Surgery, Ohio State University College of Medicine, Columbus.
Pheochromocytoma is a rare adrenal medullary tumor of chromaffin cell
origin that presents a syndrome of alpha- and beta-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.
