Unilateral versus bilateral adrenalectomy in multiple endocrine neoplasia IIA

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.

Estimated Risk of Pheochromocytoma Recurrence After Adrenal-Sparing Surgery in Patients With Multiple Endocrine Neoplasia Type 2A

“…Data on recurrent pheochromocytoma after adrenalsparing adrenalectomy were given in 15 studies. 3,7,[9][10][11][12][13][14][15][16][17][18]20,22,23 Fifty-eight (31%) of 187 patients developed a recurrent pheochromocytoma in either the ipsilateral or contralateral adrenal remnant. The interval to developing recur- rent disease was a median of 83.5 months (range, 1-375 months).…”

Bilateral Adrenalectomy with Autotransplantation of Adrenocortical Tissue or Unilateral Adrenalectomy: Treatment Options for Pheochromocytomas in Multiple Endocrine Neoplasia Type 2A.

“…Some authors have no experience of malignant pheochromocytomas [6,7]; however, van Heerden et al reported 3 cases of metastatic pheochromocytoma in their 17 patients [5], and there is an another report of a malignant case [13]. Thompson et al detected metastatic pheochromocytomas in 2 out of 8 patients with MEN 2A using 131J MIBG scans [14].…”

“…Hypertension remained in three patients, though they underwent total adrenalectomy in either one-or two-stage operation. [5,11]; however, others have expressed a different opinion about the prophylactic removal of a macroscopically normal adrenal at the same time of operation for resecting obvious pheochromocytoma on the other side [6][7][8]. Tibblin et al have recommended unilateral adrenalectomy when the largest diameter of the biggest tumor is less than 5 cm and the contralateral side is grossly normal by palpation [6].…”

“…[5,11]; however, others have expressed a different opinion about the prophylactic removal of a macroscopically normal adrenal at the same time of operation for resecting obvious pheochromocytoma on the other side [6][7][8]. Tibblin et al have recommended unilateral adrenalectomy when the largest diameter of the biggest tumor is less than 5 cm and the contralateral side is grossly normal by palpation [6]. Laimore, et al proposed that the treatment of choice for patients with unilateral pheochromocytoma is resection of only the involved gland [8].…”

Subtotale Adrenalektomie beim MEN2-Phäochromozytom

“…Hauptargument der überwiegend vom angloamerikanischen Raum ausgehenden Strategie der bilateral totalen Adrenalektomie war das krankheitsimmanente Rezidivrisiko. Nach ersten Publikationen zur funktionserhaltenden Adrenalektomie durch Tibblin [2] …”