BACKGROUND: The combination of the severity of pediatric-onset inflammatory bowel disease (IBD) phenotypes and the need for intense medical treatment may increase the risk of malignancy and mortality, but evidence regarding the extent of the problem is scarce. Therefore, the Porto Pediatric IBD working group of ESPGHAN conducted a multinational-based survey of cancer and mortality in pediatric IBD. METHODS: A survey among pediatric gastroenterologists of 20 European countries and Israel on cancer and/or mortality in the pediatric patient population with IBD was undertaken. One representative from each country repeatedly contacted all pediatric gastroenterologists from each country for reporting retrospectively cancer and/or mortality of pediatric patients with IBD after IBD onset, during 2006-2011. RESULTS: We identified 18 cases of cancers and/or 31 deaths in 44 children (26 males) who were diagnosed with IBD (ulcerative colitis, n = 21) at a median age of 10.0 years (inter quartile range, 3.0-14.0). Causes of mortality were infectious (n = 14), cancer (n = 5), uncontrolled disease activity of IBD (n = 4), procedure-related (n = 3), other non-IBD related diseases (n = 3), and unknown (n = 2). The most common malignancies were hematopoietic tumors (n = 11), of which 3 were hepatosplenic T-cell lymphoma and 3 Ebstein-Barr virus-associated lymphomas. CONCLUSIONS: Cancer and mortality in pediatric IBD are rare, but cumulative rates are not insignificant. Mortality is primarily related to infections, particularly in patients with 2 or more immunosuppressive agents, followed by cancer and uncontrolled disease. At least 6 lymphomas were likely treatment-associated by virtue of their phenotype.

Department of Pediatric Gastroenterology The Erasmus MC Sophia Children's Hospital Rotterdam The Netherlands; 2Department of Pediatric Gastroenterology Shaare Zedek Medical Center The Hebrew University of Jerusalem Jerusalem Israel; 3Department of Pediatric Gastroenterology Child Life and Health University of Edinburgh Edinburgh United Kingdom; 4Department of Pediatric Gastroenterology Dr v Hauner Children's Hospital University of Munich Medical Centre Munich Germany; 5Department of Pediatric Gastroenterology Unidad para el Cuidado Integral de la Enfermedad Inflamatoria Intestinal Pediátrica Sección de Gastroenterología Hepatología y Nutrición Pediatrica Hospital Sant Joan de Déu Barcelona Spain; 6Department of Pediatric Gastroenterology Centre for Clinical Research Västmanland Hospital Västerås; 7Karolinska Institutet Stockholm Sweden; 8Department of Pediatric Gastroenterology Bristol Royal Hospital for Children Bristol United Kingdom; 9Department of Pediatrics Gastroenterology and Nutrition Polish American Children's Hospital Jagiellonian University Medical College Cracow Poland; 10Pediatric Day Care Unit Department of Pediatrics Bnai Zion Medical Center Haifa Israel; 11 1st Department of Paediatrics Athens University Athens Greece; 12Department of Pediatric Gastroenterology 2nd Faculty of Medicine Charles University and University Hospital Motol Prague Czech Republic; 13Department of Pediatric Gastroenterology Iuliu Hatieganu University of Medicine and Pharmacy 2nd Pediatric Clinic Children's Emergency Hospital Cluj Napoca Romania; 14Pediatric Gastroenterology and Liver Unit La Sapienza University Rome Italy; 15Department of Pediatric Gastroenterology Semmelweis University Budapest Hungary; 16Department of Pediatric Gastroenterology Université Paris Descartes Sorbonne Paris Cité APHP Hôpital Necker Enfants Malades Paris France; 17Department of Pediatric Gastroenterology Refer

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Management of paediatric ulcerative colitis, part 1: Ambulatory care-An updated evidence-based consensus guideline from the European Society of Paediatric Gastroenterology, Hepatology and Nutrition and the European Crohn's and Colitis Organisation