Adrenal Neoplasia and Hyperplasia

Thirty-five percent to 40% of MEN 1 patients harbor adrenocortical lesions, and these are clearly overrepresented in the MEN 1 syndrome.15"17 Most lesions are hyperplastic, bilateral, and nonfunctioning. Aldosterone- and cortisol-secreting adenomas, however, have been reported.15"17 Hypercortisolism in MEN 1 can be the result of an ACTH-secreting pituitary process, a cortisol-secreting adenoma/ carcinoma or, rarely, due to an adrenocorticotropic hormone (ACTH)- or corticotropin-releasing factor-producing islet cell tumor or thymic carcinoid.25 Adrenocortical carcinomas have also been described in MEN 1 patients, most often in association with insulin-producing islet cell tumors.1725172 This raises the possibility of a shared underlying genetic cause or a trophic effect of insulin on adrenocortical cells.

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