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Biology and targeted therapy of gastrointestinal stromal tumors

Biology and targeted therapy of gastrointestinal stromal tumors

Tumor Research 40: 1-11

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Activating mutations of KIT or PDGF receptor alpha (PDGFRA) have been identified in the vast majority of GISTs. Most GISTs are sporadic and some kindred of familiar GIST with a germline mutation in KIT or PDGFRA have been reported. The inhibition of KIT or PDGFRA activity by the kinase inhibitor imatinib frequently results in dramatic clinical responses in advanced cases of GIST.

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