Management of Pheochromocytomas in Patients with Multiple Endocrine Neoplasia Type 2 Syndromes

Abstract

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 the Addisonian state after bilateral adrenalectomy.

High penetrance of pheochromocytoma in multiple endocrine neoplasia 2 caused by germ line RET codon 634 mutation in Japanese patients

“…To date, reports concerning MEN2 PHEO have mainly originated from Europe and the United States, and the guidelines and consensus statements have been based on those studies (4,5,9,10). Some reported a founder effect in the particular country (11).…”

Multiple endocrine neoplasia 2A syndrome presenting as peripartum cardiomyopathy due to catecholamine excess

“…Some advocate prophylactic bilateral adrenalectomy in patients with unilateral pheochromocytomas that occur as part of the MEN-2 syndrome. However, it has been found that unilateral adrenalectomy and close monitoring of symptoms and biochemical changes may be safer given that bilateral adrenalectomy poses the risk of a permanent Addisonian state (30). Because our patient's right adrenal appeared normal on intra-operative examination, it was decided that total adrenalectomy would not be performed and she would be closely monitored.…”

“…In addition, the tumor has an increased production of epinephrine, with an increased urinary epinephrine as well as an increased ratio of epinephrine to norepinephrine (29). Finally in MEN-2 patients who have a unilateral pheochromocytoma, the unaffected adrenal gland has a 50% chance of developing pheochromocytoma in 10 years (16,30).…”

“…However, the increased uptake may be seen in any situation of adrenal medullary hyperfunction, including adrenal medullary hyperplasia. Microscopic adrenal hyperplasia occurs in MEN-2A and -2B patients, both adjacent to the pheochromocytoma and also in the contralateral adrenal gland, although it may never develop into a tumor (30,33). Some advocate prophylactic bilateral adrenalectomy in patients with unilateral pheochromocytomas that occur as part of the MEN-2 syndrome.…”

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

“…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].…”