PMID- 32756962 OWN - NLM STAT- MEDLINE DCOM- 20210908 LR - 20211223 IS - 1945-7197 (Electronic) IS - 0021-972X (Print) IS - 0021-972X (Linking) VI - 106 IP - 2 DP - 2021 Jan 23 TI - Patients With MEN1 Are at an Increased Risk for Venous Thromboembolism. PG - e460-e468 LID - 10.1210/clinem/dgaa501 [doi] AB - BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited disorder predisposing the development of multiple functional and nonfunctional neuroendocrine tumors (NETs). Only uncommon MEN1-associated functional NETs such as glucagonomas (<1%) and adenocorticotropic hormone-producing tumors (<5%) are known to be associated with hypercoagulability. It is unknown if patients with MEN1 generally have an increased risk of venous thromboembolism (VTE). METHODS: We queried a prospective natural history study of germline mutation-positive MEN1 patients (n = 286) between 1991 and 2019 for all lifetime events of VTE. The search terms were: DVT, thromb, embol, PE, pulmonary embolism, clot, hematology consult, anticoagulant, coumadin, lovenox, xarelto, warfarin, aspirin, rivaroxaban, and apixaban. Incidence rates were calculated, accounting for age and sex. Comparisons were made to published incidence rates in healthy populations, different types of cancer, and Cushing's syndrome. RESULTS: Thirty-six subjects (median age 45 years, range 16-75) experienced a VTE event, yielding a prevalence rate of 12.9%. The age-sex adjusted incidence rate of VTE is 9.11 per 1000 patient-years, with a sex-adjusted lifetime incidence rate of 2.81 per 1000 patient-years. MEN1-associated lifetime incidence rates are ~2-fold higher than the estimated annual incidence rate in the general population and are comparable to the known risk in the setting of various types of cancer. Approximately 80% of patients who had a VTE were diagnosed with pancreatic NETs, of which 24% were insulinomas. Fourteen patients (42%) experienced perioperative VTE events. CONCLUSIONS: MEN1 patients have an increased risk of VTE. Further mechanistic investigation and validation from other MEN1 cohorts are needed to confirm the increased prevalence of VTE in MEN1. CI - Published by Oxford University Press on behalf of the Endocrine Society 2020. FAU - Lee, Maya E AU - Lee ME AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Ortega-Sustache, Yashira M AU - Ortega-Sustache YM AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Agarwal, Sunita K AU - Agarwal SK AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Tepede, Aisha AU - Tepede A AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Welch, James AU - Welch J AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Mandl, Adel AU - Mandl A AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Bansal, Rashika AU - Bansal R AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Tirosh, Amit AU - Tirosh A AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. AD - Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. FAU - Piaggi, Paolo AU - Piaggi P AD - Phoenix Epidemiology and Clinical Research Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Phoenix, Arizona. FAU - Cochran, Craig AU - Cochran C AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Simonds, William F AU - Simonds WF AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Weinstein, Lee S AU - Weinstein LS AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. FAU - Blau, Jenny E AU - Blau JE AD - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland. LA - eng PT - Historical Article PT - Journal Article PT - Research Support, N.I.H., Intramural PL - United States TA - J Clin Endocrinol Metab JT - The Journal of clinical endocrinology and metabolism JID - 0375362 RN - 0 (Anticoagulants) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Anticoagulants/therapeutic use MH - Female MH - History, 20th Century MH - History, 21st Century MH - Humans MH - Incidence MH - Male MH - Middle Aged MH - Multiple Endocrine Neoplasia Type 1/complications/drug therapy/*epidemiology MH - Population Surveillance MH - Prevalence MH - Retrospective Studies MH - Risk Factors MH - United States/epidemiology MH - Venous Thromboembolism/*epidemiology/etiology/prevention & control MH - Young Adult PMC - PMC7823242 OTO - NOTNLM OT - MEN1 OT - pancreatic neuroendocrine OT - venous thromboembolism EDAT- 2020/08/07 06:00 MHDA- 2021/09/09 06:00 PMCR- 2021/08/05 CRDT- 2020/08/07 06:00 PHST- 2020/05/15 00:00 [received] PHST- 2020/08/01 00:00 [accepted] PHST- 2020/08/07 06:00 [pubmed] PHST- 2021/09/09 06:00 [medline] PHST- 2020/08/07 06:00 [entrez] PHST- 2021/08/05 00:00 [pmc-release] AID - 5881385 [pii] AID - dgaa501 [pii] AID - 10.1210/clinem/dgaa501 [doi] PST - ppublish SO - J Clin Endocrinol Metab. 2021 Jan 23;106(2):e460-e468. doi: 10.1210/clinem/dgaa501.