Surgical management of the adrenal glands in the multiple endocrine neoplasia type II syndrome

During a 30-year period (1951–1981), seventeen patients underwent bilateral adrenalectomy for established adrenal medullary disease with catecholamine excess. Fourteen patients had the MEN IIa syndrome and 3 had the MEN IIb syndrome. There was no major operative morbidity and no operative mortality. One patient died 23 months after initial operation because of metastatic pheochromocytoma. The remaining patients were followed for a mean of 129 months and all were personally interviewed. Three patients had 7 uneventful pregnancies. Twelve patients underwent 23 other surgical procedures requiring general anesthesia during the followup period without any morbidity. Two patients have metastatic pheochromocytoma; 3 have clinical metastatic medullary thyroid carcinoma; 5 patients have high calcitonin values and 6 patients are in excellent health at intervals of 15, 27, 58, 110, 134, and 373 months following resection. The need for adrenal replacement therapy has not caused significant problems in any patient. The results of this study confirm that bilateral total adrenalectomy is a safe modality for the treatment of adrenal medullary disease in this syndrome and that it does not produce significant long-term morbidity.