关键词: CD117 DOG1 GEIS GIST Imatinib KIT PDGFRA Regorafenib Sunitinib

Mesh : Antineoplastic Agents / therapeutic use Combined Modality Therapy Gastrointestinal Neoplasms / diagnosis pathology surgery therapy Gastrointestinal Stromal Tumors / diagnosis secondary surgery therapy Humans Imatinib Mesylate / therapeutic use Indoles / therapeutic use Molecular Targeted Therapy Phenylurea Compounds / therapeutic use Proto-Oncogene Proteins c-kit / genetics Pyridines / therapeutic use Pyrroles / therapeutic use Sunitinib

来  源:   DOI:10.1016/j.ctrv.2016.11.011

Abstract:
Gastrointestinal stromal sarcomas (GISTs) are the most common mesenchymal tumours originating in the digestive tract. They have a characteristic morphology, are generally positive for CD117 (c-kit) and are primarily caused by activating mutations in the KIT or PDGFRA genes(1). On rare occasions, they occur in extravisceral locations such as the omentum, mesentery, pelvis and retroperitoneum. GISTs have become a model of multidisciplinary work in oncology: the participation of several specialties (oncologists, pathologists, surgeons, molecular biologists, radiologists…) has forested advances in the understanding of this tumour and the consolidation of a targeted therapy, imatinib, as the first effective molecular treatment in solid tumours. Following its introduction, median survival of patients with advanced or metastatic GIST increased from 18 to more than 60months. Sunitinib and Regorafenib are two targeted agents with worldwide approval for second- and third-line treatment, respectively, in metastatic GIST.
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