1. Gastroenteropancreatic Neuroendocrine Neoplasms in Patients with Inflammatory Bowel Disease: An ECCO CONFER Multicentre Case Series
- Author
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Stefano Festa, Giulia Zerboni, Lauranne A A P Derikx, Davide Giuseppe Ribaldone, Gabriele Dragoni, Christianne Buskens, Els Nieveen van Dijkum, Daniela Pugliese, Francesco Panzuto, Iwona Krela-Kaźmierczak, Hilla Reiss Mintz, Ariella Bar-Gil Shitrit, Marìa Chaparro, Javier P Gisbert, Uri Kopylov, Niels Teich, Elez Vainer, Iris Nagtegaal, Frank Hoentjen, Maria Jose Garcia, Rafal Filip, Kalliopi Foteinogiannopoulou, Ioannis E Koutroubakis, Marjorie Argollo, Roy L J van Wanrooij, Hendrik Laja, Triana Lobaton, Marie Truyens, Tamas Molnar, Edoardo Savarino, Annalisa Aratari, Claudio Papi, Idan Goren, Surgery, CCA - Cancer Treatment and Quality of Life, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, and Gastroenterology and hepatology
- Subjects
Male ,Crohn’s disease ,Inflammatory bowel disease ,neuroendocrine neoplasms ,ulcerative colitis ,Female ,Humans ,Middle Aged ,Inflammatory Bowel Diseases ,Intestinal Neoplasms ,Neuroendocrine Tumors ,Pancreatic Neoplasms ,Stomach Neoplasms ,crohn’s disease ,Gastroenterology ,General Medicine ,digestive system diseases ,Crohn's disease ,Tumours of the digestive tract Radboud Institute for Molecular Life Sciences [Radboudumc 14] ,03.02. Klinikai orvostan ,Inflammatory diseases Radboud Institute for Molecular Life Sciences [Radboudumc 5] - Abstract
Background Gastroenteropancreatic neuroendocrine neoplasms [GEP-NENs] have rarely been reported in association with inflammatory bowel diseases [IBDs]. Methods An ECCO COllaborative Network For Exceptionally Rare case reports project [ECCO-CONFER] collects cases of GEP-NENs diagnosed in patients with IBD. Results GEP-NEN was diagnosed in 100 IBD patients; 61% female, 55% Crohn’s disease, median age 48 years (interquartile range [IQR] 38-59]). The most common location was the appendix [39%] followed by the colon [22%]. Comprehensive IBD-related data were available for 50 individuals with a median follow-up of 30 months [IQR 11-70] following NEN diagnosis. Median duration of IBD at NEN diagnosis was 84 months [IQR 10-151], and in 18% of cases NEN and IBD were diagnosed concomitantly. At diagnosis, 20/50 were stage-I [T1N0M0], and 28/50 were graded G1 [ki67 ≤2%]. Incidental diagnosis of NEN and concomitantly IBD diagnosis were associated with an earlier NEN stage [p = 0.01 and p = 0.02, respectively]. Exposure to immunomodulatory or biologic therapy was not associated with advanced NEN stage or grade. Primary GEP-NEN were more frequently found in the segment affected by IBD [62% vs 38%]. At the last follow-up data, 47/50 patients were alive, and only two deaths were related to NEN. Conclusions In the largest case series to date, prognosis of patients with GEP-NEN and IBD seems favourable. Incidental NEN diagnosis correlates with an earlier NEN stage, and IBD-related therapies are probably independent of NEN stage and grade. The association of GEP-NEN location and the segment affected by IBD may suggest a possible role of inflammation in NEN tumorigenesis
- Published
- 2022