Gastroenteropancreatic Neuroendocrine Neoplasms in Patients with Inflammatory Bowel Disease: An ECCO CONFER Multicentre Case Series

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 GarciaRafal 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

Research output: Contribution to journalArticleAcademicpeer-review


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.
Original languageEnglish
Pages (from-to)940-945
Number of pages6
JournalJournal of Crohn's & colitis
Issue number6
Publication statusPublished - 1 Jun 2022

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