Multiple Endocrine Neoplasia Type 1 and the Pancreas: Diagnosis and Treatment of Functioning and Non-Functioning Pancreatic and Duodenal Neuroendocrine Neoplasia within the MEN1 Syndrome – An International Consensus Statement
Year: 2020
Type: article
Abstract: The better understanding of the biological behavior of multiple endocrine neoplasia type 1 (MEN1) organ manifestations and the increase in clinical experience warrant a revision of previously published guidelines. Duodenopancreatic neuroendocrine neoplasias (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 reevaluate recommendations for their diagnosis and treatment. Especially over the last 2 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 syndrome in an effort to further standardize and improve treatment and follow-up, as well as to establish 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 guidelines of 2001 and 2012 and attempts to supplement the recommendations issued by various national and international societies.
Source: Neuroendocrinology
DOI: 10.1159/000511791
Authors: Bruno Niederle, Andreas Selberherr, Detlef K. Bartsch, Maria L. Brandi, Gerard M. Doherty, Massimo Falconi, Pierre Goudet, Thorvardur R. Halfdanarson, Tetsuhide Ito, Robert T. Jensen, Alberto Larghi, Lingaku Lee, Kjell Öberg, Marianne Pavel, Aurel Perren, Samira M. Sadowski, Francesco Tonelli, Frédéric Triponez, Gerlof D. Valk, Dermot O’Toole, David Scott-Coombes, Rajesh V. Thakker, Geoffrey B. Thompson, Giorgio Treglia, Bertram Wiedenmann
Institutions: Medical University of Vienna, Philipps University of Marburg, University of Florence, Brigham and Women's Hospital, Harvard University, Vita-Salute San Raffaele University, CHU Dijon Bourgogne, Mayo Clinic, Mayo Clinic Comprehensive Cancer Center (Minnesota), Fukuoka Sanno Hospital, International University of Health and Welfare, National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases, Università Cattolica del Sacro Cuore, Agostino Gemelli University Polyclinic, Istituti di Ricovero e Cura a Carattere Scientifico, Uppsala University Hospital, Universitätsklinikum Erlangen, University of Bern, National Cancer Institute, University Hospital of Geneva, University Medical Center Utrecht, Trinity College Dublin, St. Vincent's University Hospital, St. James's Hospital, University Hospital of Wales, Churchill Hospital, University of Oxford, Oxford Centre for Diabetes, Endocrinology and Metabolism, Ente Ospedaliero Cantonale, Istituto Imaging della Svizzera Italiana, Charité – Universitätsmedizin Berlin
Language: English
Subfield: Epidemiology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W3088779642