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

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) collecting 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 (IQR 38-59)]. The most common location was the appendix (39%) followed by the colon (22%). Comprehensive IBD-related data was 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 graded G1 (ki67≤2%). Incidental diagnosis of NEN and concomitantly with 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 favorable. 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 tumourigenesis

Mucinous and Signet-Ring Cell Colonic Adenocarcinoma in Inflammatory Bowel Disease: A Case–Control Study

“…In our IBD study population, the frequency of mucinous/signet-ring cell adenocarcinoma localized in colorectal segments involved by IBD was high (70.6%), although comparable to the frequency observed in patients with standard adenocarcinoma (56.5%). Recently, a multicenter study reported the possible association between neuroendocrine neoplasms and inflamed segments in IBD [27]. The same was not observed in the present study, which focused on colorectal adenocarcinoma.…”

Extraintestinal Cancers in Inflammatory Bowel Disease: A Literature Review

“…Both CD and UC lead to chronic inflammation of the gut, but inflammation may also affect other organs, causing extraintestinal manifestations [3][4][5][6]. Moreover, both conditions are associated with impaired quality of life and increased disability [7][8][9].…”

“…Chronic inflammatory insults of the gut may increase the risk of cancer formation in patients with IBD, such as colorectal cancer [7][8][9]. Risk factors for the development of intestinal cancers are well known, including clinical characteristics of the patients or phenotypes of the disease, and there are precise indications for screening, surveillance, and management of these patients [7].…”