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

• Published in: Journal of Crohn's and colitis Vol. 16; no. 6; pp. 940 - 945
• Main Authors: Festa, Stefano, Zerboni, Giulia, Derikx, Lauranne A A P, Ribaldone, Davide Giuseppe, Dragoni, Gabriele, Buskens, Christianne, van Dijkum, Els Nieveen, Pugliese, Daniela, Panzuto, Francesco, Krela-Kaźmierczak, Iwona, Mintz, Hilla Reiss, Shitrit, Ariella Bar-Gil, Chaparro, Marìa, Gisbert, Javier P, Kopylov, Uri, Teich, Niels, Vainer, Elez, Nagtegaal, Iris, Hoentjen, Frank, Garcia, Maria Jose, Filip, Rafal, Foteinogiannopoulou, Kalliopi, Koutroubakis, Ioannis E, Argollo, Marjorie, van Wanrooij, Roy L J, Laja, Hendrik, Lobaton, Triana, Truyens, Marie, Molnar, Tamas, Savarino, Edoardo, Aratari, Annalisa, Papi, Claudio, Goren, Idan
• Format: Journal Article
• Language: English
• Published: UK Oxford University Press 14.07.2022
• Subjects: Inflammatory bowel disease; neuroendocrine neoplasms; ulcerative colitis; Crohn’s disease
• ISSN: 1873-9946; 1876-4479
• DOI: 10.1093/ecco-jcc/jjab217

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