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      Clinicopathological features and menin expression of pancreatic neuroendocrine neoplasm associated with multiple endocrine neoplasia type 1

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          Abstract

          Background/Purpose

          We examined therapeutic strategies for pancreatic neuroendocrine neoplasm (pNEN) associated with MEN1 (M‐pNEN) by investigating clinicopathological features and menin expression.

          Methods

          Seventy‐seven patients who underwent resection of pNEN at our department from January 2001 to December 2017 were retrospectively analyzed. Immunohistochemical analysis of menin was performed using resected specimens.

          Results

          Seven patients (9%) met the diagnostic criteria for MEN1. M‐pNEN had more tumors ( P < .01), a higher recurrence rate ( P = .028), and higher residual pancreatic recurrence ( P < .01) than sporadic pNEN (S‐pNEN). There were no significant differences in tumor size, lymph node metastasis, or World Health Organization grade between the two groups. Reduced menin staining in the tumor nuclei was found in 86% of M‐pNEN; whereas only 34% of S‐pNEN showed decreased nuclear staining. The remainder (66%) showed strong nuclear staining similar to normal islet cells ( P = .0071). Furthermore, four patients (57%) with MEN1 had many microadenomas with reduced nuclear menin staining. Overall survival of M‐pNEN patients was significantly better than S‐pNEN patients ( P = .049).

          Conclusion

          M‐pNEN patients tend to develop spatially and temporally multifocal pNENs. However, M‐pNEN patient prognosis is good with repeated surgeries at recurrence. Therefore, minimal resection with strict follow‐up is recommended rather than extensive pancreatic resections for consideration of recurrence in M‐pNEN.

          Abstract

          This study aimed to describe therapeutic strategies for pancreatic neuroendocrine neoplasm associated with multiple endocrine neoplasia type 1 (M‐pNEN) by investigating the clinicopathological features and menin expression. Sonoda and colleagues concluded that minimal resection with strict follow‐up is recommended rather than extensive pancreatic resections for consideration of recurrence in M‐pNEN.

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          Most cited references17

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          One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States.

          PURPOSE Neuroendocrine tumors (NETs) are considered rare tumors and can produce a variety of hormones. In this study, we examined the epidemiology of and prognostic factors for NETs, because a thorough examination of neither had previously been performed. METHODS The Surveillance, Epidemiology, and End Results (SEER) Program registries were searched to identify NET cases from 1973 to 2004. Associated population data were used for incidence and prevalence analyses. Results We identified 35,618 patients with NETs. We observed a significant increase in the reported annual age-adjusted incidence of NETs from 1973 (1.09/100,000) to 2004 (5.25/100,000). Using the SEER 9 registry data, we estimated the 29-year limited-duration prevalence of NETs on January 1, 2004, to be 9,263. Also, the estimated 29-year limited-duration prevalence in the United States on that date was 103,312 cases (35/100,000). The most common primary tumor site varied by race, with the lung being the most common in white patients, and the rectum being the most common in Asian/Pacific Islander, American Indian/Alaskan Native, and African American patients. Additionally, survival duration varied by histologic grade. In multivariate analysis of patients with well-differentiated to moderately differentiated NETs, disease stage, primary tumor site, histologic grade, sex, race, age, and year of diagnosis were predictors of outcome (P < .001). CONCLUSION We observed increased reported incidence of NETs and increased survival durations over time, suggesting that NETs are more prevalent than previously reported. Clinicians need to be become familiar with the natural history and patterns of disease progression, which are characteristic of these tumors.
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            ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors

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              Guidelines for diagnosis and therapy of MEN type 1 and type 2.

              This is a consensus statement from an international group, mostly of clinical endocrinologists. MEN1 and MEN2 are hereditary cancer syndromes. The commonest tumors secrete PTH or gastrin in MEN1, and calcitonin or catecholamines in MEN2. Management strategies improved after the discoveries of their genes. MEN1 has no clear syndromic variants. Tumor monitoring in MEN1 carriers includes biochemical tests yearly and imaging tests less often. Neck surgery includes subtotal or total parathyroidectomy, parathyroid cryopreservation, and thymectomy. Proton pump inhibitors or somatostatin analogs are the main management for oversecretion of entero-pancreatic hormones, except insulin. The roles for surgery of most entero-pancreatic tumors present several controversies: exclusion of most operations on gastrinomas and indications for surgery on other tumors. Each MEN1 family probably has an inactivating MEN1 germline mutation. Testing for a germline MEN1 mutation gives useful information, but rarely mandates an intervention. The most distinctive MEN2 variants are MEN2A, MEN2B, and familial medullary thyroid cancer (MTC). They vary in aggressiveness of MTC and spectrum of disturbed organs. Mortality in MEN2 is greater from MTC than from pheochromocytoma. Thyroidectomy, during childhood if possible, is the goal in all MEN2 carriers to prevent or cure MTC. Each MEN2 index case probably has an activating germline RET mutation. RET testing has replaced calcitonin testing to diagnose the MEN2 carrier state. The specific RET codon mutation correlates with the MEN2 syndromic variant, the age of onset of MTC, and the aggressiveness of MTC; consequently, that mutation should guide major management decisions, such as whether and when to perform thyroidectomy.
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                Author and article information

                Contributors
                hdobaba@kumamoto-u.ac.jp
                Journal
                J Hepatobiliary Pancreat Sci
                J Hepatobiliary Pancreat Sci
                10.1002/(ISSN)1868-6982
                JHBP
                Journal of Hepato-Biliary-Pancreatic Sciences
                John Wiley and Sons Inc. (Hoboken )
                1868-6974
                1868-6982
                14 May 2020
                December 2020
                : 27
                : 12 ( doiID: 10.1002/jhbp.v27.12 )
                : 984-991
                Affiliations
                [ 1 ] Department of Gastroenterological Surgery Graduate School of Life Sciences Kumamoto University Kumamoto Japan
                [ 2 ] Department of Metabolic Medicine Faculty of Life Sciences Kumamoto University Kumamoto Japan
                Author notes
                [*] [* ] Correspondence

                Hideo Baba, Department of Gastroenterological Surgery, Kumamoto University Graduate School of Medical Sciences, 1‐1‐1 Honjo, Kumamoto 860‐8556, Japan.

                Email: hdobaba@ 123456kumamoto-u.ac.jp

                Author information
                https://orcid.org/0000-0002-6610-3075
                https://orcid.org/0000-0001-8439-6660
                https://orcid.org/0000-0002-7880-889X
                Article
                JHBP739
                10.1002/jhbp.739
                7818135
                32278331
                1e0bcd64-7a1a-40a4-9017-1bb82050f870
                © 2020 The Authors. Journal of Hepato‐Biliary‐Pancreatic Sciences published by John Wiley & Sons Australia, Ltd on behalf of Japanese Society of Hepato-Biliary-Pancreatic Surgery

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                : 23 December 2019
                : 02 February 2020
                : 02 March 2020
                Page count
                Figures: 5, Tables: 2, Pages: 8, Words: 3756
                Categories
                Original Article
                Original Articles
                Custom metadata
                2.0
                December 2020
                Converter:WILEY_ML3GV2_TO_JATSPMC version:5.9.6 mode:remove_FC converted:21.01.2021

                Gastroenterology & Hepatology
                menin,multiple endocrine neoplasia type 1,pancreatic neuroendocrine neoplasm

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