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SHR Neuro Krebs Kardio Lipid Stoffw Microb

Benesch, M; Wardelmann, E; Leuschner, I; Koscielniak, E; .
Gastrointestinal stromal tumors in childhood and adolescence.
MONATSSCHR KINDERHEILK. 2012; 160(10): 967-+. Doi: 10.1007/s00112-012-2692-7
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Führende Autor*innen der Med Uni Graz
Benesch Martin
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Abstract:
Gastrointestinal stromal tumors (GISTs) are extremely rare among children and differ clinically and biologically from adult GISTs. The characteristic molecular genetic alterations of these neoplasms, i.e. oncogenic mutations of the receptor tyrosine kinase genes KIT and platelet-derived growth factor receptor alpha (PDGFRA) making them amenable to targeted therapy by receptor tyrosine kinase inhibitors, are uncommon in pediatric GISTs. Standards for the management of GISTs in children and adolescents do not presently exist. The nationwide registration of these patients within the soft tissue sarcoma register of the Cooperative Weichteilsarkom Studiengruppe (SoTiSaR) is a prerequisite to increase the knowledge about these rare tumors and to develop appropriate management strategies.

Find related publications in this database (Keywords)
Gastrointestinal stromal tumors
Children
Adolescents
Carney triad
Carney-Stratakis syndrome
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