Malignancy and mortality in pediatric patients with inflammatory bowel disease: A multinational study from the porto pediatric IBD group

Lissy De Ridder, Dan Turner, David C. Wilson, Sibylle Koletzko, Javier Martin-De-Carpi, Ulrika L. Fagerberg, Christine Spray, Malgorzata Sladek, Ron Shaoul, Eleftheria Roma-Giannikou, Jiri Bronsky, Daniela E. Serban, Salvatore Cucchiara, Gabor Veres, Frank M. Ruemmele, Iva Hojsak, Kaija L. Kolho, Ieuan H. Davies, Marina Aloi, Paolo LionettiGigi Veereman-Wauters, Christian P. Braegger, Eunice Trindade, Anne V. Wewer, Almuthe Hauer, Arie Levine

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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.

Original languageEnglish
Pages (from-to)291-300
Number of pages10
JournalInflammatory bowel diseases
Volume20
Issue number2
DOIs
Publication statusPublished - Feb 1 2014

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Keywords

  • Cancer
  • Mortality
  • Pediatric inflammatory bowel disease
  • Survey

ASJC Scopus subject areas

  • Immunology and Allergy
  • Gastroenterology

Cite this

De Ridder, L., Turner, D., Wilson, D. C., Koletzko, S., Martin-De-Carpi, J., Fagerberg, U. L., Spray, C., Sladek, M., Shaoul, R., Roma-Giannikou, E., Bronsky, J., Serban, D. E., Cucchiara, S., Veres, G., Ruemmele, F. M., Hojsak, I., Kolho, K. L., Davies, I. H., Aloi, M., ... Levine, A. (2014). Malignancy and mortality in pediatric patients with inflammatory bowel disease: A multinational study from the porto pediatric IBD group. Inflammatory bowel diseases, 20(2), 291-300. https://doi.org/10.1097/01.MIB.0000439066.69340.3c