Pediatric Colorectal Carcinoma is Associated With Excellent Outcome in the Context of Cancer Predisposition Syndromes

Weber M, Schneider DT, Offenmüller S, Kaatsch P, Einsiedel HG, Benesch M, Claviez A, Ebinger M, Kramm C, Kratz C, Lawlor J, Leuschner I, Merkel S, Metzler M, Nustede R, Petsch S, Seeger KH, Schlegel PG, Suttorp M, Zolk O, Brecht IB (2016)

Publication Type: Journal article

Publication year: 2016

Journal

Book Volume: 63

Pages Range: 611-7

Journal Issue: 4

DOI: 10.1002/pbc.25839

Abstract

Colorectal carcinoma (CRC) is the second most common adult cancer in Germany, however, it is extremely rare in children and adolescents. In these patients, previous literature describes aggressive behavior and diagnosis at advanced stage.Thirty-one patients with CRC age <= 18 years and treated between 1990 and 2012 have been identified through the structures and registries of the German Society for Pediatric Oncology and Hematology.The age range was 9-18 years (median 13.5 years); the median follow-up time was 43.9 months (range 1-124 months). Twenty-six patients (84%) were tested for a genetic tumor syndrome (GTS); of these, 11 patients (35% of all patients) tested positive (eight cases of Lynch syndrome, one patient with familial adenomatous polyposis, two patients with constitutional mismatch repair deficiency). An unfavorable histology was reported in 55% of the records (n = 17), a poor differentiation (grade III) in 68% of carcinoma (n = 21). Overall survival (OS) and event-free survival at 5 years was 52.0% and 65.6%, respectively. Five-year survival according to stage was 100% in stage II (n = 2), 100% in stage III (n = 13), and 12.9% in stage IV (n = 15; P < 0.001). Five-year OS in patients with and without a defined GTS was 100% and 36.5% (P = 0.019), respectively.Children and adolescents with CRC are frequently diagnosed in advanced stages and have an unfavorable prognosis. In this study, a high percentage of pediatric CRC patients presented with a tumor predisposition syndrome and showed an especially favorable OS.

Authors with CRIS profile

Involved external institutions

Universitätsmedizin der Johannes Gutenberg-Universität Mainz Germany (DE) Universitätsklinikum Carl Gustav Carus Dresden Germany (DE) Universität Ulm Germany (DE) Universitätsklinikum Würzburg Germany (DE) Charité - Universitätsmedizin Berlin Germany (DE) Klinikum der Medizinischen Hochschule Hannover Germany (DE) Christian-Albrechts-Universität zu Kiel Germany (DE) Universitätsklinikum Göttingen Germany (DE) Universitätsklinikum Tübingen Germany (DE) Universitätsklinikum Schleswig-Holstein (UKSH) Germany (DE) Medizinische Universität Graz Austria (AT) Universitätsmedizin Greifswald / Universitätsklinikum Greifswald Germany (DE) Klinikum Dortmund Germany (DE)

How to cite

APA:

Weber, M., Schneider, D.T., Offenmüller, S., Kaatsch, P., Einsiedel, H.G., Benesch, M.,... Brecht, I.B. (2016). Pediatric Colorectal Carcinoma is Associated With Excellent Outcome in the Context of Cancer Predisposition Syndromes. Pediatric Blood & Cancer, 63(4), 611-7. https://doi.org/10.1002/pbc.25839

MLA:

Weber, Marie, et al. "Pediatric Colorectal Carcinoma is Associated With Excellent Outcome in the Context of Cancer Predisposition Syndromes." Pediatric Blood & Cancer 63.4 (2016): 611-7.

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