Gastrointestinal stromal tumors in childhood and adolescence

被引：0
作者：
Benesch, M. [1 ]
Wardelmann, E. [2 ]
Leuschner, I. [3 ]
Koscielniak, E. [4 ]
机构：
[1] Med Univ Graz, Univ Klin Kinder & Jugendheilkunde, Klin Abt Padiat Hamatoonkol, A-8036 Graz, Austria
[2] Univ Klinikum Bonn, Inst Pathol, Bonn, Germany
[3] Univ Klinikum Schleswig Holstein, Zent Kindertumorregister, Kiel, Germany
[4] Klinikum Stuttgart, Olgahosp, Stuttgart, Germany
来源：
MONATSSCHRIFT KINDERHEILKUNDE | 2012年 / 160卷 / 10期
关键词：
Gastrointestinal stromal tumors; Children; Adolescents; Carney triad; Carney-Stratakis syndrome; TERM-FOLLOW-UP; OF-THE-LITERATURE; CARNEY TRIAD; PULMONARY CHONDROMA; ADJUVANT IMATINIB; GERMLINE MUTATION; YOUNG-ADULTS; KIT; PARAGANGLIOMA; MESYLATE;
D O I：
10.1007/s00112-012-2692-7
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
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.
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页码：967 / +
页数：7
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