P124 Gastroenteropancreatic Neuroendocrine Neoplasms in patients with Inflammatory Bowel Disease: An ECCO CONFER Multicentre Case Series

Festa, S.(1);Zerboni, G.(2);Derikx, L.(3);Ribaldone, D.G.(4);Dragoni, G.(5);Buskens, C.J.(6);Nieveen van Dijkum, E.(6);Pugliese, D.(7);Panzuto, F.(8);Krela-Kaźmierczak, I.(9);Reiss, H.(10);Shitrit, A.B.G.(11);Chaparro, M.(12);Gisbert, J.P.(12);Kopylov, U.(13);Teich, N.(14);Vainer, E.(15);Nagtegaal, I.(16);Hoentjen, F.(3);Garcia, M.J.(17);Filip, R.(18);Foteinogiannopoulou, K.(19);Koutroubakis, I.(19);Argollo, M.(20);van Wanrooij, R.(21);Laja, H.(22);Truyens, M.(23);Lobaton Ortega, T.(23);Molnar, T.(24);Savarino, E.(25);Aratari, A.(1);Papi, C.(1);Goren, I.(10);

(1)San Filippo Neri Hospital, IBD Unit, Rome, Italy;(2)Nuovo Ospedale dei Castelli, Division of Gastroenterology, Rome, Italy;(3)Radboud University Medical Center, Inflammatory Bowel Disease Center- Department of Gastroenterology and Hepatology, Nijmegen, The Netherlands;(4)University of Turin, Department of Medical Sciences- Division of Gastroenterology, Turin, Italy;(5)Careggi University Hospital, IBD Referral Center- Department of Gastroenterology-, Florence, Italy;(6)Amsterdam University Medical Hospitals, location AMC, Amsterdam, The Netherlands;(7)Fondazione Policlinico Universitario "A. Gemelli"- IRCCS, CEMAD - IBD UNIT - Unità Operativa Complessa di Medicina Interna e Gastroenterologia- Dipartimento di Scienze Mediche e Chirurgiche, Rome, Italy;(8)Sant'Andrea University Hospital- ENETS Center of Excellence of Rome, Digestive Diseases Unit, Rome, Italy;(9)Human Nutrition and Internal Diseases- Poznan University- of Medical Sciences-, Department of Gastroenterology, Poznan, Poland;(10)Rabin Medical Center- Petah Tikva- Israel. Affiliated to the Sackler Faculty of Medicine- Tel Aviv University, IBD unit- Division of Gastroenterology, Tel Aviv, Israel;(11)Faculty of Medicine- Hebrew University of Jerusalem- Digestive diseases institute, IBD MOM unit- Digestive diseases institute- Shaare Zedek Medical Center, Jerusalem, Israel;(12)Hospital Universitario de La Princesa- Instituto de Investigación Sanitaria Princesa- Universidad Autónoma de Madrid- Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas, Gastroenterology Unit, Madrid, Spain;(13)Tel-HaShomer Sheba Medical Center- Ramat Gan- Israel- and Sackler Medical School, Department of Gastroenterology, Tel Aviv, Israel;(14)Gastroenterological Practice, Gastroenterology Unit, Leipzig, Germany;(15)Hadassah-Hebrew University Medical Center, Department of Gastroenterology, Jerusalem, Israel;(16)Radboud University Medical Center, Department of Pathology, Nijmegen, The Netherlands;(17)Hospital Universitario Marqués de Valdecilla- IDIVAL, Department of Gastroenterology, Santander, Spain;(18)University of Rzeszow, Department of Gastroenterology, Rzeszow, Poland;(19)University Hospital of Heraklion, Department of Gastroenterology, Crete, Greece;(20)D'OR Institute of Research and Education IDOR, Department of Gastroenterology, Sao Paulo, Brazil;(21)Amsterdam UMC- Vrije Universiteit Amsterdam- AGEM Research Institute, Department of Gastroenterology and Hepatology, Amsterdam, The Netherlands;(22)Tartu University Hospital, Department of Gastroenterology, Tartu, Estonia;(23)University Hospital Ghent- Belgium UZ Ghent, Department of Gastroenterology, Ghent, Belgium;(24)University of Szeged, Department of Gastroenterology, Szeged, Poland;(25)University of Padua, Division of Gastroenterology- Department of Surgery- Oncology and Gastroenterology, Padua, Italy


Neuroendocrine Neoplasms (NENs) are a heterogeneous group of tumours deriving from the diffuse endocrine system. NENs may occur almost everywhere in the body but are most common in the gastrointestinal tract, the pancreas, and the lungs, with gastroenteropancreatic (GEP) tumours representing 70% of all NENs. GEP-NENs have rarely been reported in association with inflammatory bowel diseases (IBDs) but no definitive relationship between these tumours and IBD has been established


This was an ECCO COllaborative Network For Exceptionally Rare case reports project (ECCO-CONFER). We included cases of GEP-NENs diagnosed in patients with IBD that met the diagnostic criteria for NEN according to the European Neuroendocrine Tumour Society. Data were retrospectively collected in a standardized case report form and analysed for event association with patient's and IBD-related factors


GEP-NEN was diagnosed in 100 patients with IBD [61% female, 55% Crohn's disease, median age 48 years (IQR 37-59)]. Overall the most common location was the appendix (39/100) followed by the colon (22/100). Complete IBD-related data was available for 50 individuals with a median follow-up of 30.5 months (IQR 11.2-70) following NEN diagnosis. At the last follow-up data, 47/50 patients were alive. Three deaths occurred, of which 2 were related to NEN. Median duration of IBD at NEN diagnosis was 84 months (IQR 10-151), and in 18% of cases NEN and IBD were diagnosed concomitantly. 20/50 of NENs were at stage I (T1N0M0) and 28/50 graded G1 (ki 67 ≤2 %) at diagnosis.
Incidental diagnosis of NEN either during follow-up or during surgery as well as receiving diagnosis of NEN concomitantly with IBD was significantly associated with an earlier NEN stage (p< 0.01 and p<0.02, respectively). Exposure to immunomodulatory and/or biologic therapy was not associated with advanced NEN stage or grade. Interestingly, primary GEP-NEN sites significantly correlated to the segment affected by IBD (62% vs 38% p = 0.02)


In the largest case series to date, prognosis of patients with concomitant GEP-NEN and IBD seems favorable. Incidental NEN diagnosis correlates with an earlier NEN stage and IBD-related therapies are 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