| 赵志威 朱琳 姜涛 荆琼优 王剑冰 朴大勋.直肠间质瘤临床病理学及外科治疗的研究进展[J].,2016,16(5):982-985 |
| 直肠间质瘤临床病理学及外科治疗的研究进展 |
| Progress of Clinical Pathologic and Surgical Management of Rectal Stromal Tumors |
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| DOI： |
| 中文关键词: 直肠间质瘤 c-KIT(CD117) PDGFRalpha 病理学 免疫组化 外科手术 靶向治疗 |
| 英文关键词: Rectal stromal tumors c-KIT (CD117) PDGFR-alpha Pathologic Immunohistochemical Surgery Targeted therapy |
| 基金项目:吴阶平医学基金会临床科研专项资助项目（320. 6750. 12172）；国家自然科学基金项目（30972898） |
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| 中文摘要: |
| 胃肠间质瘤（gastrointestinal stromal tumors, GISTs）是消化道最常见的间叶组织来源的肿瘤，其标志性地表达KIT 蛋白
（CD117），目前研究认为KIT 或PDGFR琢（血小板源性生长因子-alpha，platelet derived growth factor alpha）基因突变导致了GISTs 的
发生。直肠间质瘤发病率非常低且多为偶然发现，但其多数属于高危组、易复发和转移。外科手术是治疗间质瘤的最主要方式，但
酪氨酸激酶抑制剂（tyrosine kinase inhibitors, TKIs）的出现，使无法切除或者转移性GISTs 的治疗方式发生了革命性的变化。
GISTs的诊治需要包括外科、肿瘤科、病理科以及放射科等多学科协助共同完成，首先对疾病进行初期评估，术前术后的管理，而
后对预后进行随访。通过术前的靶向治疗进行减瘤，优化手术的时机，术后TKIs 规范化治疗，从而提高患者的生存率，延长其生
存时间。本文对直肠间质瘤临床病理学及外科治疗的研究进展进行综述。 |
| 英文摘要: |
| Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. They
express KIT protein (CD117) symbolically. Current study suggests that the mutations of KIT or PDGFRalpha(platelet derived growth factor
alpha) can lead to GISTs. The incidence of rectal stromal tumors is very low and found incidentally, the majority appear to be high risk
and carry a significant likelihood of recurrent and metastatic disease. Surgery remains the mainstay of treatment for primary disease. Tyrosine
Kinase inhibitors (TKIs), make revolutionary changes in the treatment of unresectable or metastatic GISTs. GISTs are best treated
by a multidisciplinary team comprised of the surgeon, medical oncologist, pathologist, and radiologist in the initial evaluation, management,
and in continued follow-up. Through the preoperative pharmacologic debulking, optimizing the timing of surgery, and postoperative
standard treating with TKIs, patients can obtain the greater survival rate and longer survival time. |
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