Copyright 2006 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2006
An ideal operation for a pheochromocytoma in a patient with multiple endocrine neoplasia type 2A (MEN 2A) would be to remove the pheochromocytoma and all of the medulla while saving the adrenal cortex. Residual medullary tissue can become hyperplastic or develop recurrent pheochromocytoma. The cortex is necessary for adrenocortical steroid production, and insufficient cortex causes Addison disease. The adrenal gland is like a peanut butter sandwich: the medulla is the peanut butter and the cortex, the bread. The ideal operation of removing all the peanut butter (medulla) but keeping the bread (cortex) is a nearly impossible task. In practice, cortex-sparing adrenalectomy is a compromise, weighing the risks of recurrent pheochromocytoma and adrenocortical insufficiency.
Duh Q. Estimated Risk of Pheochromocytoma Recurrence After Adrenal-Sparing Surgery in Patients With Multiple Endocrine Neoplasia Type 2A—Invited Critique. Arch Surg. 2006;141(12):1205. doi:10.1001/archsurg.141.12.1205