Adrenal disease is present in 10%–73% of MEN1 patients on imaging studies23,48,128,228,374,388,390,453 and is reported to cause MEN1-related deaths in occasional series (0.4%,150 4%465) and case reports.161,385 Adrenal tumors in MEN1 patients are reported to cause a number of functional hormone excess syndromes including Cushing syndrome, hyperaldosteronism, pheochromocytoma, androgen secretion, or feminization.8,27,48,57,128,157,228,261,276,385,388,390,436,453 Functional hormone excess states caused by the adrenal tumors are rarely a cause of MEN1-related death, because in most series >90% of the adrenal lesions are not associated with a functional syndrome (except 1 series228 where 38% with adrenal lesions had Cushing syndrome).48,388,390 In various series,23,48,128,140,157,374,385,390,453 0–6% of MEN1 patients develop adrenal cortical carcinomas, which can be aggressive, invasive, and associated with a functional syndrome such as Cushing syndrome, and which can contribute to or cause death in these patients. However, in both our series including the 106 NIH MEN1/ZES patients and the 227 MEN1/PET patients from the pooled literature, no patient died of adrenal disease, similar to what was reported in a number of recent large MEN1 series,23,78,129,157,217,448 supporting the conclusion that it is an uncommon cause of death at present in MEN1 patients. This conclusion is also supported by the results from the 758-patient GTE study of MEN1 patients. In that study, those with adrenal tumors had a trend toward an increased risk of death compared to unaffected patients, but it did not reach significance (p = 0.064) (HR = 1.72; 95% CI, 0.97–3.06).150