| 野生型胃肠道间质瘤的研究进展 |
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| 引用本文： | 白怡冰,杨佳妮,方琳,张艳桥. 野生型胃肠道间质瘤的研究进展[J]. 实用肿瘤学杂志, 2020, 34(6): 524-529. DOI: 10.11904/j.issn.1002-3070.2020.06.008 |
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| 作者姓名： | 白怡冰 杨佳妮 方琳 张艳桥 |
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| 作者单位： | 哈尔滨医科大学附属肿瘤医院(哈尔滨 150081) |
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| 基金项目： | 国家自然科学基金项目(编号:81672428);国家自然科学基金项目(编号:81872427);黑龙江省应用技术研究与开发计划(编号:GA19C002) |
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| 摘 要： | 胃肠道间质瘤(GIST)是最常见的消化道间叶组织来源肿瘤,约10%的GIST患者分子检测无KIT/PDGFRA基因突变,称为野生型GIST。根据是否有琥珀酸脱氢酶B(SDHB)表达缺失,野生型GIST可分为SDH缺陷型和非SDH缺陷型,SDH缺陷型包括无综合征相关性、Carney三联征相关性及Carney-stratakis综合征相关性GIST;非SDH缺陷型包括BRAF突变、Ⅰ型神经纤维瘤病相关性、K/N-RAS突变及四重野生型GIST等。野生型GIST的发生发展、临床病理特征和治疗原则均与KIT或PDGFRA突变的GIST有较大差异。本文就野生型GIST的分子机制和临床诊疗进展做一综述。
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| 关 键 词： | 野生型胃肠道间质瘤 琥珀酸脱氢酶 诊断 治疗 |
| 收稿时间： | 2020-02-22 |
Research progress of wild-type gastrointestinal stromal tumors |
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| BAI Yibing,YANG Jiani,FANG Lin,ZHANG Yanqiao. Research progress of wild-type gastrointestinal stromal tumors[J]. Journal of Practical Oncology, 2020, 34(6): 524-529. DOI: 10.11904/j.issn.1002-3070.2020.06.008 |
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| Authors: | BAI Yibing YANG Jiani FANG Lin ZHANG Yanqiao |
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| Affiliation: | Harbin Medical University Cancer Hospital,Harbin 150081,China |
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| Abstract: | Gastrointestinal stromal tumor(GIST)is the most common tumor of archenteric mesenchymal tissue.About 10% of patients are diagnosed as GIST morphologically and immunohistochemically without KIT and PDGFRA gene mutations which is defined wild-type GIST.According to the absence of succinate dehydrogenase B(SDHB)expression,wild-type GIST can be divided into SDH-deficient and non-SDH-deficient types.SDH-deficient types include syndrome-free associated,Carney triad associated,and Carney-stratakis synthesis associated GIST;non-SDH-deficient types include BRAF mutations,type I neurofibromatosis-related,K/N-RAS mutations,and quadruple wild-type GIST.The occurrence,development,clinicopathological features and treatment principles of wild-type GIST are significantly different from those of KIT or PDGFRA mutant GIST.This article reviews the molecular mechanism and clinical progress of wild-type GIST. |
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| Keywords: | Wild-type gastrointestinal stromal tumor Succinate dehydrogenase(SDH) Diagnosis Treatment |
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