Abstract: Research progress of succinate dehydrogenase-deficient gastrointestinal stromal tumors        YANG Shu-ya，YE Ying-jiang，GAO Zhi-dong. Department of Gastroenterological Surgery, Laboratory of Surgical Oncology, Peking University People's Hospital, Beijing 100044, China
Corresponding author：GAO Zhi-dong, E-mail:gaozhidong@pkuph.edu.cn
Abstract    Succinate dehydrogenase（SDH）-deficient gastrointestinal stromal tumor（GIST）is a type of wild-type GIST without KIT/PDGFRA mutation，and it is characterized by negative SDHB immunohistochemistry.SDH deficiency leads to the accumulation of succinic acid in cells.The latter，as a kind of oncometabolite，can trigger a variety of epigenetic and metabolic changes，thus promoting tumor occurrence and development.The age of onset of SDH-deficient GIST is often early，and the lesion is mainly located in the stomach，which sometimes can be associated with paraganglioma and/or pulmonary chondroma.This type of GIST is still primarily treated with surgery.It does not respond well to targeted drugs therapy，while traditional chemotherapy drugs such as temozolomide may potentially affect it. SDH-deficient GIST's pathogenesis and effective treatment methods need to be further explored.

Key words: gastrointestinal stromal tumor, succinate dehydrogenase

摘要： 琥珀酸脱氢酶（SDH）缺陷型胃肠间质瘤（GIST）是一类没有KIT/PDGFRA突变的野生型GIST，以SDHB免疫组化阴性为特征。SDH缺陷导致细胞内琥珀酸堆积，后者作为促癌代谢物可引发多种表观遗传学和代谢改变，促进肿瘤发生发展。SDH缺陷型GIST发病年龄早，病变部位以胃为主，可合并发生副神经节瘤或肺软骨瘤。该型GIST以手术治疗为主，对靶向治疗反应欠佳，替莫唑胺等传统化疗药物可能对其有潜在疗效。SDH缺陷型GIST的发病机制及有效治疗方法等仍有待进一步探索。

关键词: 胃肠间质瘤, 琥珀酸脱氢酶