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Gastrointestinal Stromal Tumors

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Rare Tumors In Children and Adolescents

Part of the book series: Pediatric Oncology ((PEDIATRICO))

Abstract

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract in adults but are very uncommon in children and adolescents. The exact incidence of GIST in this population has not been determined. In the three largest GIST series to date, the percentage of patients below the age of 21 ranged from 0.5% to 2.7%. There is a reported annual incidence of 6.5–14.5 per million cases of GIST overall, while the UK National Registry of Childhood Tumors has reported an annual incidence of 0.02 per million cases of GIST in children below the age of 14 years (Stiller 2007; Benesch et al. 2009).

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References

  • Agaram NP, Laquaglia MP, Ustun B et al (2008) Molecular characterization of pediatric gastrointestinal stromal tumors. Clin Cancer Res 14:3204–3215

    Article  PubMed  CAS  Google Scholar 

  • Benesch M, Wardelmann E, Ferrari A et al (2009) Gastrointestinal stromal tumors (GIST) in children and adolescents: a comprehensive review of the current literature. Pediatr Blood Cancer 53:1171–1179

    Article  PubMed  Google Scholar 

  • Benesch M, Leuschner I, Wardelmann E, et al (2011) Gastrointestinal stromal tumours in children and young adults: a clinicopathologic series with long-term follow-up from the database of the Cooperative Weichteilsarkom Studiengruppe (CWS). Eur J Cancer 47(11):1692–1698, Epub 2011 Apr 10

    Article  PubMed  Google Scholar 

  • Carney JA, Stratakis CA (2002) Familial paraganglioma and gastric stromal sarcoma: a new syndrome distinct from the Carney triad. Am J Med Genet 108:132–139

    Article  PubMed  Google Scholar 

  • Carney JA, Sheps SG, Go VL et al (1977) The triad of gastric leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma. N Engl J Med 296:1517–1518

    Article  PubMed  CAS  Google Scholar 

  • Casali PG, Jost L, Reichardt P et al (2010) Gastrointestinal stromal tumors: ESMO Clinical Recommendations for diagnosis, treatment and follow-up. Ann Oncol 21(Suppl 5):ii98–102

    Article  PubMed  Google Scholar 

  • Corless CL, Heinrich MC (2008) Molecular pathobiology of gastrointestinal stromal sarcomas. Annu Rev Pathol Mech Dis 3:557–586

    Article  CAS  Google Scholar 

  • Demetri GD, Benjamin RS, Blanke CD et al (2007) NCCN Task Force report: optimal management of patients with gastrointestinal stromal tumors (GIST) – update of the NCCN clinical practice guidelines. J Natl Compr Canc Netw 5(Suppl 2):S1–S29

    PubMed  Google Scholar 

  • Hirota S, Isozaki K, Moriyama Y et al (1998) Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 279(5350):577–580

    Article  PubMed  CAS  Google Scholar 

  • Janeway KA, Liegl B, Harlow B et al (2007) Pediatric KIT–wild-type and platelet-derived growth factor receptor alpha–wild-type gastrointestinal stromal tumors share KIT activation but not mechanisms of genetic progression with adult gastrointestinal stromal tumors. Cancer Res 67:9084–9088

    Article  PubMed  CAS  Google Scholar 

  • Janeway KA, Albritton KH, Van Den Abbeele AD et al (2009) Sunitinib treatment of pediatric metastatic GIST after failure of imatinib. Pediatr Blood Cancer 52:767–771

    Article  PubMed  Google Scholar 

  • Kindblom LG, Remotti HE, Aldenborg F et al (1998) Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 152:1259–1269

    PubMed  CAS  Google Scholar 

  • Kuroiwa M, Hiwatari M, Hirato J et al (2005) Advanced-stage gastrointestinal stromal tumor treated with imatinib in a 12-year-old girl with a unique mutation of PDGFRA. J Pediatr Surg 40:1798–1801

    Article  PubMed  Google Scholar 

  • Mazur MT, Clark HB (1983) Gastric stromal tumors. Reappraisal of histogenesis. Am J Surg Pathol 7:507–519

    Article  PubMed  CAS  Google Scholar 

  • McWhinney SR, Pasini B, Stratakis CA (2007) Familial gastrointestinal stromal tumors and germ-line mutations. N Engl J Med 357:1054–1056

    Article  PubMed  CAS  Google Scholar 

  • Miettinen M, Lasota J, Sobin LH (2005) Gastrointestinal stromal tumors of the stomach in children and young adults: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases with long-term follow-up and review of the literature. Am J Surg Pathol 29:1373–1381

    Article  PubMed  Google Scholar 

  • Nishida T, Hirota S, Taniguchi M et al (1998) Familial gastrointestinal stromal tumours with germline mutation of the KIT gene. Nat Genet 19:323–324

    Article  PubMed  CAS  Google Scholar 

  • Stiller C (2007) Childhood cancer in Britain: incidence, survival and mortality. Oxford University Press, New York, p. 104

    Google Scholar 

  • Takazawa Y, Sakurai S, Sakuma Y et al (2005) Gastrointestinal stromal tumors of neurofibromatosis type I (von Recklinghausen’s disease). Am J Surg Pathol 29:755–763

    Article  PubMed  Google Scholar 

  • Tarn C, Rink L, Merkel E et al (2008) Insulin-like growth factor 1 receptor is a potential therapeutic target for gastrointestinal stromal tumors. Proc Natl Acad Sci USA 105:8387–8392

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Auenbruggerplatz 38, 8036, Graz, Austria

    M. Benesch

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  1. M. Benesch
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Correspondence to M. Benesch .

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Editors and Affiliations

  1. Klinik für Kinder- und Jugendmedizin, Klinikum Dortmund gGmbH, Beurhausstr. 40, Dortmund, 44137, Germany

    Dominik T. Schneider

  2. Klinik für Kinder und Jugendliche, Universitätsklinikum Erlangen-Nürnberg, Loschgestr. 15, Erlangen, 91054, Germany

    Ines B. Brecht

  3. at Egleston, Dept. Pediatric Hematology/Oncology, Children's Healthcare of Atlanta, Uppergate Drive NE., 2015, Atlanta, 30322, Georgia, USA

    Thomas A. Olson

  4. Unità di Oncologia Pediatrica, Istituto Nazionale Tumori, Via G. Venezian 1, Milano, 20133, Italy

    Andrea Ferrari

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© 2012 Springer-Verlag Berlin Heidelberg

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Benesch, M. (2012). Gastrointestinal Stromal Tumors. In: Schneider, D., Brecht, I., Olson, T., Ferrari, A. (eds) Rare Tumors In Children and Adolescents. Pediatric Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-04197-6_34

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  • DOI: https://doi.org/10.1007/978-3-642-04197-6_34

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-04196-9

  • Online ISBN: 978-3-642-04197-6

  • eBook Packages: MedicineMedicine (R0)

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