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
Niederle, Bruno; Selberherr, Andreas; Bartsch, Detlef K.; Brandi, Maria L.; Doherty, Gerard M.; Falconi, Massimo; Goudet, Pierre; Halfdanarson, Thorvardur R.; Ito, Tetsuhide; Jensen, Robert T.; Larghi, Alberto; Lee, Lingaku; Öberg, Kjell; Pavel, Marianne; Perren, Aurel; Sadowski, Samira M.; Tonelli, Francesco; Triponez, Frédéric; Valk, Gerlof D.; O'Toole, Dermot; Scott-Coombes, David; Thakker, Rajesh V.; Thompson, Geoffrey B.; Treglia, Giorgio; Wiedenmann, Bertram
(2021) Neuroendocrinology, volume 111, issue 7, pp. 609 - 630
(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
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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.
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Keywords: Consensus statement, Diagnosis, Multiple endocrine neoplasia type 1, Neuroendocrine pancreatic tumors, Treatment, Pancreatic Neoplasms/diagnosis, Humans, Duodenal Neoplasms/diagnosis, Consensus, Multiple Endocrine Neoplasia Type 1/diagnosis, Endocrine and Autonomic Systems, Endocrinology, Cellular and Molecular Neuroscience, Endocrinology, Diabetes and Metabolism, Review, Journal Article
ISSN: 0028-3835
Publisher: S. Karger AG
Note: Funding Information: The project was supported by the European Society of Endocrine Surgeons (ESES) and by the European Neuroendocrine Tumor Society (ENETS). The authors wish to thank Daniela Larwa and Karl Thomanek for their services in preparing the final version of this consensus statement. We are also grateful to Karl Thomanek for linguistic review and proofreading. Publisher Copyright: © 2020
(Peer reviewed)