title: Malignancy and mortality in pediatric patients with inflammatory bowel disease: a multinational study from the porto pediatric IBD group.
abstract: 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.
authors.affiliation: 1Department 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; 11First Department of Paediatrics, Athens University, Athens, Greece; 12Department of Pediatric Gastroenterology, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic; 13Department of Pediatric Gastroenterology, "Iuliu Hatieganu" University of Medicine and Pharmacy, Second 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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mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005060:Europe / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D015212:Inflammatory Bowel Diseases / Q000150:complications* / Q000453:epidemiology; D008297:Male; D009369:Neoplasms / Q000209:etiology* / Q000401:mortality*; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends