Clinical study of pheochromocytoma.

Seventeen patients underwent 22 operations for pheochromocytoma over a 26 year period. The patients included 11 in three kindreds of familial pheochromocytoma and 6 with sporadic tumors. The diagnosis was usually suggested by the patient's history. Confirmation of the diagnosis frequently required repeated testing for urinary or plasma catecholamines. Arteriography was the most effective method of preoperative tumor localization. Two tumors were proven nonfunctional. Others secreted either norepinephrine or epinephrine and norepinephrine. The familial patients showed a high incidence of multiple, bilateral and extraadrenal lesions. In contrast to previous reports, the familial tumors were found more often in the right than in the left adrenal gland. HL-A genotyping analysis of the sporadic and familial patients failed to demonstrate any HL-A antigen association or interdependent segregation of tumor and HL-A antigens within kindreds.