MANAGEMENT OF PHEOCHROMOCYTOMAS IN PATIENTS WITH MULTIPLE ENDOCRINE NEOPLASIA TYPE-2 SYNDROMES

Objective The authors sought to determine the optimal surgical managem ent of pheochromocytomas that develop in patients with multiple endocr ine neoplasia (MEN) type 2 syndromes. Summary Background data The perf ormance of empirical bilateral adrenalectomy in patients with MEN 2A o r MEN 2B, whether or not they have bilateral pheochromocytomas, is con troversial. Methods The results of unilateral or bilateral adrenalecto my were studied in 58 patients (49 with MEN 2A and 9 with MEN 2B). Rec urrence of disease was evaluated by measuring 24-hour urinary excretio n rates of catecholamines and metabolites and by computed tomography ( CT) scanning. Results The mean postoperative follow-up was 9.40 years. There was no operative mortality and malignant or extra-adrenal pheoc hromocytomas were not present. Twenty-three patients with a unilateral pheochromocytoma and a macroscopically normal contralateral gland und erwent unilateral adrenalectomy. A pheochromocytoma developed in the r emaining gland a mean of 11.87 years after the primary adrenalectomy i n 12 (52%) patients. Conversely, 11 (48%) patients did not develop a p heochromocytoma during a mean interval of 5.18 years. In the interval after unilateral adrenalectomy, no patient experienced hypertensive cr ises or other complications related to an undiagnosed pheochromocytoma . Ten (23%) of 43 patients having both adrenal glands removed (either at a single operation or sequentially) experienced at least one episod e of acute adrenal insufficiency or Addisonian crisis, including one p atient who died during a bout of influenza. Conclusions Based on these data, the treatment of choice for patients with MEN 2A or MEN 2B and a unilateral pheochromocytoma is resection of only the involved gland. Substantial morbidity and significant mortality are associated with t he Addisonian state after bilateral adrenalectomy.