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本文引用的文献

1
Remarkable effects of imatinib in a family with young onset gastrointestinal stromal tumors and cutaneous hyperpigmentation associated with a germline KIT-Trp557Arg mutation: case report and literature overview.伊马替尼对一个患有年轻发病的胃肠道间质瘤和与种系KIT-Trp557Arg突变相关的皮肤色素沉着的家族具有显著疗效:病例报告及文献综述
Fam Cancer. 2018 Apr;17(2):247-253. doi: 10.1007/s10689-017-0024-8.
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Diagnostic and treatment strategy for small gastrointestinal stromal tumors.小胃肠间质瘤的诊断与治疗策略
Cancer. 2016 Oct 15;122(20):3110-3118. doi: 10.1002/cncr.30239. Epub 2016 Aug 1.
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Gastrointestinal Stromal Tumours: Review of 150 Cases from a Single Centre.胃肠道间质瘤:来自单一中心的150例病例回顾
Indian J Surg. 2015 Dec;77(Suppl 2):505-10. doi: 10.1007/s12262-013-0899-z. Epub 2013 Mar 28.
4
Clinical presentations of gastric small gastrointestinal stromal tumors mimics functional dyspepsia symptoms.胃小胃肠道间质瘤的临床表现类似功能性消化不良症状。
World J Gastroenterol. 2014 Sep 7;20(33):11800-7. doi: 10.3748/wjg.v20.i33.11800.
5
A histopathological and immunohistochemical approach of surgical emergencies of GIST. An interdisciplinary study.胃肠道间质瘤外科急症的组织病理学和免疫组织化学研究方法。一项跨学科研究。
Rom J Morphol Embryol. 2014;55(2 Suppl):619-27.
6
Contrast-enhanced endoscopic ultrasonography can predict a higher malignant potential of gastrointestinal stromal tumors by visualizing large newly formed vessels.对比增强内镜超声检查可通过观察大量新形成的血管来预测胃肠道间质瘤具有更高的恶性潜能。
J Clin Ultrasound. 2015 Feb;43(2):89-97. doi: 10.1002/jcu.22195. Epub 2014 Jul 16.
7
Gastrointestinal stromal tumors (GIST) related emergencies.胃肠道间质瘤(GIST)相关急症。
Int J Surg. 2014;12(4):269-80. doi: 10.1016/j.ijsu.2014.02.004. Epub 2014 Feb 12.
8
Gastrointestinal stromal tumors.胃肠道间质瘤。
Gastroenterol Clin North Am. 2013 Jun;42(2):399-415. doi: 10.1016/j.gtc.2013.01.001. Epub 2013 Mar 13.
9
Gastrointestinal stromal tumour.胃肠道间质瘤。
Lancet. 2013 Sep 14;382(9896):973-83. doi: 10.1016/S0140-6736(13)60106-3. Epub 2013 Apr 24.
10
A subset of gastrointestinal stromal tumors previously regarded as wild-type tumors carries somatic activating mutations in KIT exon 8 (p.D419del).一组以前被认为是野生型肿瘤的胃肠道间质瘤携带 KIT 外显子 8 中的体细胞激活突变(p.D419del)。
Mod Pathol. 2013 Jul;26(7):1004-12. doi: 10.1038/modpathol.2013.47. Epub 2013 Apr 19.

胃肠道间质瘤的临床表现

Clinical Presentation of Gastrointestinal Stromal Tumors.

作者信息

Menge Franka, Jakob Jens, Kasper Bernd, Smakic Armand, Gaiser Timo, Hohenberger Peter

机构信息

Division of Surgical Oncology, Mannheim University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany.

Department of Surgery, Mannheim University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany.

出版信息

Visc Med. 2018 Oct;34(5):335-340. doi: 10.1159/000494303. Epub 2018 Oct 13.

DOI:10.1159/000494303
PMID:30498699
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6257088/
Abstract

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. They constitute 1-2% of all gastrointestinal neoplasms but are the most common subtype of soft tissue sarcomas, accounting for 20-25%. In the late 1990s, GISTs were more and more recognized as a particular tumor entity. The tumors are supposed to originate from the interstitial pacemaker cells of Cajal. They are usually well circumscribed and can be located in every part of the tubular gastrointestinal tract. Most often GISTs occur in the stomach, followed by the small bowel and colon/rectum. In contrast to epithelial tumors, GISTs grow transmurally and submucosal. GISTs can be found with highly variable growth features including tumors with intraluminal, intra- or transmural, and pedunculated appearance. Here we describe the most common clinical presentation of GISTs on the basis of our 809 patients managed from 2004 to 2017. The median age of our patients was 59 years and the average size of GIST was 75 mm (range: 4 mm to 35 cm). The clinical presentation is very heterogeneous, depending on tumor site, size, and growth pattern. GISTs of the stomach is the group with the lowest rate of acute or emergency symptoms with 31%, followed by GISTs of the duodenum with 42%, whereas GISTs of the small bowel show acute symptoms in more than 50% of the cases and have an emergency surgery rate of almost 15%. Many patients are diagnosed accidentally, through screening examinations, or with latent, unspecific symptoms.

摘要

胃肠道间质瘤(GISTs)是胃肠道最常见的间叶性肿瘤。它们占所有胃肠道肿瘤的1%-2%,却是软组织肉瘤最常见的亚型,占20%-25%。在20世纪90年代后期,GISTs越来越被认为是一种特殊的肿瘤实体。这些肿瘤被认为起源于 Cajal 间质起搏细胞。它们通常边界清晰,可位于管状胃肠道的各个部位。GISTs最常发生于胃,其次是小肠和结肠/直肠。与上皮性肿瘤不同,GISTs呈跨壁和黏膜下生长。GISTs可表现出高度可变的生长特征,包括腔内、壁内或跨壁以及带蒂外观的肿瘤。在此,我们根据2004年至2017年管理的809例患者描述GISTs最常见的临床表现。我们患者的中位年龄为59岁,GIST的平均大小为75mm(范围:4mm至35cm)。临床表现非常异质性,取决于肿瘤部位、大小和生长方式。胃GISTs出现急性或紧急症状的比例最低,为31%,其次是十二指肠GISTs,为42%,而小肠GISTs在超过50%的病例中出现急性症状,急诊手术率近15%。许多患者是通过筛查检查意外诊断出来的,或者是有潜在的、非特异性症状。