Mesh : Humans Gastrointestinal Stromal Tumors / diagnosis genetics therapy Receptor, Platelet-Derived Growth Factor alpha / genetics Proto-Oncogene Proteins c-kit / genetics Mutation

来  源:   DOI:10.6004/jnccn.2022.0058   PDF(Pubmed)

Abstract:
Gastrointestinal stromal tumors (GIST) are the most common type of soft tissue sarcoma that occur throughout the gastrointestinal tract. Most of these tumors are caused by oncogenic activating mutations in the KIT or PDGFRA genes. The NCCN Guidelines for GIST provide recommendations for the diagnosis, evaluation, treatment, and follow-up of patients with these tumors. These NCCN Guidelines Insights summarize the panel discussion behind recent important updates to the guidelines, including revised systemic therapy options for unresectable, progressive, or metastatic GIST based on mutational status, and updated recommendations for the management of GIST that develop resistance to specific tyrosine kinase inhibitors.
摘要:
胃肠道间质瘤(GIST)是在整个胃肠道中发生的最常见类型的软组织肉瘤。这些肿瘤中的大多数是由KIT或PDGFRA基因中的致癌激活突变引起的。GIST的NCCN指南为诊断提供了建议,评估,治疗,以及这些肿瘤患者的随访。这些NCCN指南见解总结了指南最近重要更新背后的小组讨论,包括修改后的不可切除的全身治疗方案,进步,或基于突变状态的转移性GIST,并更新了对特定酪氨酸激酶抑制剂产生耐药性的GIST的管理建议。
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