Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

• DOI: 10.1097/md.0b013e3182954af1
• Publication Date: 2013-05-03T13:12:46Z
• Authors: Tetsuhide Ito; Hisato Igarashi; Hirotsugu Uehara; Marc J. Berna; Robert T. Jensen
• Keywords: MEN1; Endocrine disease; Zollinger-Ellison syndrome

Multiple endocrine neoplasia type 1 (MEN1) is classically characterized by the development of functional or nonfunctional hyperplasia tumors in tissues (parathyroid, pancreas, pituitary, adrenal). Because effective treatments have been developed for hormone excess state, which was a major cause death these patients past, coupled with recognition that nonendocrine increasingly develop late disease course, natural history has changed. An understanding current causes important to tailor treatment and help identify prognostic factors; however, it generally lacking. To add our understanding, we conducted detailed analysis factors from prospective long-term National Institutes Health (NIH) study 106 MEN1 pancreatic Zollinger-Ellison syndrome (MEN1/ZES patients) compared results those pooled literature data 227 (MEN1/PET reported case reports small series, 1386 large series. In NIH series over mean follow-up 24.5 years, 24 (23%) died (14 MEN1-related 10 non-MEN1-related deaths). Comparing found no acute complications due acid hypersecretion, 8%–14% other causes, similar published since 1995. 2 (the series), two-thirds an one-third cause, agrees values literature, although varied widely. main deaths were malignant nature PETs, followed thymic carcinoid tumors. These differ number especially before 1990s. The decreasing frequency, cardiovascular disease, > lung diseases, cerebrovascular diseases. most frequent tumor colorectal, renal breast, oropharyngeal. Although both overall disease-related survival are better than past (30-yr series: 82% overall, 88% disease-related), age at 55 younger expected general population. Detailed correlated clinical, laboratory, characteristics allowed identification factors. Poor included higher fasting gastrin levels, presence hormonal syndromes, need >3 parathyroidectomies, liver metastases distant metastases, aggressive PET growth, new lesions. this helped define present, enabled us should be helpful tailoring short- management, as well directing research efforts determining present.