The progressive fragmentation of the KIT/PDGFRA wild-type (WT) gastrointestinal stromal tumors (GIST)

被引:49
|
作者
Nannini, Margherita [1 ]
Urbini, Milena [2 ]
Astolfi, Annalisa [2 ]
Biasco, Guido [1 ,2 ]
Pantaleo, Mara A. [1 ,2 ]
机构
[1] Univ Bologna, St Orsola Malpighi Hosp, Dept Specialized Expt & Diagnost Med, Via Massarenti 9, I-40138 Bologna, Italy
[2] Univ Bologna, Giorgio Prodi Canc Res Ctr, Bologna, Italy
关键词
GIST; SDH; Quadruple wild-type; ETV6-NTRK3; MEN1; MAX; FGFR1; SUCCINATE-DEHYDROGENASE; FUNCTION MUTATIONS; GENE-EXPRESSION; KIT; PDGFRA; SDHA; SPECTRUM; RECEPTOR; BIOLOGY; SUBSET;
D O I
10.1186/s12967-017-1212-x
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
Recent advances in molecular biology have revolutionized the concept of KIT/PDGFRA wild type (WT) gastrointestinal stromal tumors (GIST) than the past. Indeed, from being defined as GIST without KIT or PDGFRA mutations, we are now faced with the opposite scenario, where KIT/PDGFRA WT GIST are "positively" defined according to their specific molecular alterations. In particular, if until recently KIT/PDGFRA GIST without abnormalities of KIT, PDGFRA, SDH, and the RAS signaling pathway were referred as quadruple WT GIST, today also this small subset of GIST is emerging out as a group of heterogeneous distinct entities with multiple different molecular alterations. Therefore, given this still growing and rapidly evolving scenario, the progressive molecular fragmentation may inevitably lead over the time to the disappearance of KIT/PDGFRA WT GIST, destined to be singularly defined by their molecular fingerprint.
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页数:4
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