Unilateral versus bilateral adrenalectomy in multiple endocrine neoplasia IIA

Abstract

Abstract Factors influencing the result of various forms of surgical treatment for pheochromocytoma were studied in patients with multiple endocrine neoplasia IIA (MEN IIA) syndrome. The material consisted of 18 patients belonging to 4 non‐related families operated on during 1966–1981 with a mean follow‐up time of 7.4 years. Twenty‐seven adrenals were removed at 22 operations. In all adrenals, multiple pheochromocytomas and/or medullary hyperplasia were present. In 13 patients the first operation was a unilateral adrenalectomy. Four of these had to be reoperated on because of recurring or persisting symptoms. Five patients were operated on bilaterally at the first instance. In 4 of these, the reason was bilateral macroscopic tumor involvement. Various pre‐ and intraoperative findings were evaluated with respect to the surgical result. The maximum diameter of the largest tumor was the only factor relating positively to surgical success with unilateral operation. Of 9 patients who remained asymptomatic following unilateral adrenalectomy, 8 had tumors less than 5 cm in diameter. The 4 patients who had to be reoperated on with a contralateral adrenalectomy all had a maximum tumor diameter exceeding 5 cm. A pragmatic approach to the problem of timing and extent of adrenal surgery in MEN IIA is advocated. Clinical, biochemical, histopathological, genetic, and social dimensions of the disease should be taken into consideration when choosing the surgical procedure .