Multiple Endocrine Neoplasia Type 1 (MEN1) and the Pancreas - Diagnosis and Treatment of Functioning and Non-Functioning Pancreatic and Duodenal Neuroendocrine Neoplasia within the MEN1 Syndrome - An International Consensus Statement.,Neuroendocrinology

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Multiple Endocrine Neoplasia Type 1 (MEN1) and the Pancreas - Diagnosis and Treatment of Functioning and Non-Functioning Pancreatic and Duodenal Neuroendocrine Neoplasia within the MEN1 Syndrome - An International Consensus Statement.
Neuroendocrinology ( IF 3.2 ) Pub Date : 2020-09-24 , DOI: 10.1159/000511791
Bruno Niederle ₁ , Andreas Selberherr ₂ , Detlef K Bartsch ₃ , Maria L Brandi ₄ , Gerard M Doherty ₅ , Massimo Falconi ₆ , Pierre Goudet ₇ , Thorvardur R Halfdanarson ₈ , Tetsuhide Ito ₉ , Robert T Jensen ₁₀ , Alberto Larghi ₁₁ , Lingaku Lee ₁₀ , Kjell Öberg ₁₂ , Marianne Pavel ₁₃ , Aurel Perren ₁₄ , Samira M Sadowski ₁₅ , Francesco Tonelli ₁₆ , Frédéric Triponez ₁₇ , Gerlof D Valk ₁₈ , Dermot O'Toole ₁₉ , David Scott-Coombes ₂₀ , Rajesh V Thakker ₂₁ , Geoffrey B Thompson ₂₂ , Giorgio Treglia ₂₃ , Bertram Wiedenmann ₂₄

Affiliation

Department of Surgery, Medical University of Vienna, Vienna, Austria,
Department of Surgery, Medical University of Vienna, Vienna, Austria.
Department of Visceral, Thoracic and Vascular Surgery, Philipps University Marburg, Marburg, Germany.
Firmo Lab, Fondazione F.I.R.M.O. and University Florence, Florence, Italy.
Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Pancreatic Surgery, San Raffaele Scientific Institute, "Vita-Salute" University, Milan, Italy.
Service de Chirurgie Viscérale et Endocrinienne, Centre Hospitalier Universitaire François Mitterand, Dijon, France.
Department of Medical Oncology, Mayo Clinic Cancer Center, Rochester, Minnesota, USA.
Neuroendocrine Tumor Centre, Fukuoka Sanno Hospital and Department of Gastroenterology, Graduate School of Medical Sciences, International University of Health and Welfare, Sawara-ku, Fukuoka, Japan.
National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA.
Digestive Endoscopy Unit, Fondazione Policlinico A. Gemelli IRCCS and Center for Endoscopic Research, Therapeutics and Training, Catholic University, Rome, Italy.
Endocrine Oncology, Department of Medical Sciences, Uppsala University Hospital, Uppsala, Sweden.
Endocrinology and Diabetology, Department of Medicine 1, University Clinic of Erlangen, Erlangen, Germany.
Institute of Pathology, University of Bern, Bern, Switzerland.
Surgical Oncology Program, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.
Thoracic and Endocrine Surgery, University Hospital of Geneva, Geneva, Switzerland.
Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.
Department of Clinical Medicine, St. James's Hospital and St Vincent's University Hospital and Trinity College, Dublin, Ireland.
Department of Endocrine Surgery, University Hospital of Wales, Cardiff, United Kingdom.
Academic Endocrine Unit, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, United Kingdom.
Section of Endocrine Surgery, Department of Gastroenterologic and General Surgery, Mayo Clinic, Rochester, Minnesota, USA.
Imaging Institute of Southern Switzerland, Ente Ospedaliero Cantonale, Bellinzona, Switzerland.
Department of Gastroenterology and Hepatology, Campus Virchow-Klinikum and Campus Charité Mitte, Charité - Universitätsmedizin Berlin, Berlin, Germany.

The better understanding of the biological behavior of MEN1 organ manifestations and the in-crease in clinical experience warrant a revision of previously published guidelines. DP-NENs are still the second most common manifestation in MEN1 and, besides NENs of the thymus, remain a leading cause of death. DP-NENs are thus of main interest in the effort to re-evaluate recommendations for their diagnosis and treatment. Especially over the last two years, more clinical experience has documented the follow-up of treated and untreated (natural-course) DP-NENs. It was the aim of the international consortium of experts in endocrinology, genetics, radiology, surgery, gastroenterology and oncology to systematically review the literature and to present a consensus statement based on the highest levels of evidence. Reviewing the literature published over the past decade, the focus was on the diagnosis of F- and NF-DP-NENs within the MEN1 syn-drome in an effort to further standardize and improve treatment and follow-up, as well as to es-tablish a "logbook" for the diagnosis and treatment of DP-NENs. This shall help further reduce complications and improve long-term treatment results in these rare tumors. The following international consensus statement builds upon the previously published guide-lines of 2001 and 2012 and attempts to supplement the recommendations issued by various na-tional and international societies.

中文翻译：

多发性内分泌瘤 1 型 (MEN1) 和胰腺 - MEN1 综合征中功能性和非功能性胰腺和十二指肠神经内分泌瘤的诊断和治疗 - 国际共识声明。

对 MEN1 器官表现的生物学行为的更好理解和临床经验的增加需要对先前发布的指南进行修订。DP-NENs 仍然是 MEN1 中第二常见的表现，除了胸腺的 NENs 之外，它仍然是导致死亡的主要原因。因此，DP-NEN 的主要兴趣在于重新评估其诊断和治疗的建议。特别是在过去两年中，更多的临床经验记录了治疗和未治疗（自然过程）DP-NEN 的随访。国际内分泌学、遗传学、放射学、外科、胃肠病学和肿瘤学专家联盟的目标是系统地审查文献并根据最高水平的证据提出共识声明。回顾过去十年发表的文献，重点是 MEN1 综合征中 F-和 NF-DP-NEN 的诊断，以进一步规范和改善治疗和随访，以及为 DP-NENs 的诊断和治疗建立一个“日志”。这将有助于进一步减少并发症并改善这些罕见肿瘤的长期治疗效果。以下国际共识声明以先前发布的 2001 年和 2012 年指南为基础，并试图补充各种国家和国际协会发布的建议。用于DP-NENs的诊断和治疗。这将有助于进一步减少并发症并改善这些罕见肿瘤的长期治疗效果。以下国际共识声明以先前发布的 2001 年和 2012 年指南为基础，并试图补充各种国家和国际协会发布的建议。用于DP-NENs的诊断和治疗。这将有助于进一步减少并发症并改善这些罕见肿瘤的长期治疗效果。以下国际共识声明以先前发布的 2001 年和 2012 年指南为基础，并试图补充各种国家和国际协会发布的建议。

更新日期：2020-09-24

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