Gastrointestinal Stromal Tumors in the 21 st Century

摘要: Gastrointestinal stromal tumours (GISTs) are rare mesenchymal lesions accounting for only 0.2% of all gastrointestinal neoplasms. These tumors arise from the interstitial cells of Cajal, with mutations described in proto-oncogenes such as KIT, PDGFRA, DOG-1, and SDH. The majority these asymptomatic, thus true incidence remains unknown. While patients typically undergo initial endoscopy, CT scan and/or MRI, findings often nonspecific and require a biopsy to identify tumor. As such, immunohistochemical evaluation is the gold-standard accurate diagnosis GIST. Though surgical excision remains gold-standard curative management, discovery imatinib, a tyrosine kinase inhibitor (TKI), has revolutionized treatment GIST in the 21st century a “prototype” molecular targeted therapy solid tumors. Risk assessment for recurrence divides into low high-risk categories. In the latter, role adjuvant TKI confers significantly better prognosis than previously observed. However, secondary conferring drug resistance remain an ongoing challenge few alternative treatment options available intolerant/refractory to TKI therapy. this review, we summarize epidemiology, pathogenesis, clinical presentation, diagnosis, pathology features, management options, and prognostic features GISTs.