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肛周胃肠道外间质瘤

Perianal extragastrointestinal stromal tumor.

作者信息

Kang Ayoung, Cho Sung Hwan, Park Byung-Soo, Son Gyung Mo, Kim Hyun Sung, Kim Jae-Joon, Kim Su Jin, Shin Dong Hoon, Kim Tae Un

机构信息

Department of General Surgery, Pusan National University Yangsan Hospital, Yangsan, Korea.

Internal Medicine, Pusan National University Yangsan Hospital, Yangsan, Korea.

出版信息

Korean J Clin Oncol. 2020 Dec;16(2):138-141. doi: 10.14216/kjco.20021. Epub 2020 Dec 31.

DOI:10.14216/kjco.20021
PMID:36945718
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9942734/
Abstract

An extragastrointestinal stromal tumor (EGIST) is a gastrointestinal stromal tumor that arises outside of the gastrointestinal tract. Most EGISTs are located in the omentum, mesentery, and retroperitoneum. The occurrence of an EGIST at the perianal region is very rare. Herein, we report our experience with EGISTs in the perianal area and review the literature. A 70-year-old man presented to our hospital with a 2-year history of anal discomfort. A pelvic magnetic resonance imaging scan showed a homogenous, well-defined, soft tissue density mass. The patient underwent mass excision, and the pathological examination confirmed that the mass was an EGIST. The size of the tumor was 4.3×3.2 cm, and the mitotic count was 1 per 50 high-power fields. The tumor cells were immunohistochemically positive for KIT and CD34 but were negative for S-100 and alpha-smooth muscle actin. There were no other abnormal findings in the gastrointestinal tract; upon pathological review, this case was confirmed as perianal EGIST. Therefore, EGIST should be considered as a differential diagnosis of perianal masses.

摘要

胃肠道外间质瘤(EGIST)是一种起源于胃肠道外的胃肠道间质瘤。大多数EGIST位于大网膜、肠系膜和腹膜后。EGIST发生于肛周区域非常罕见。在此,我们报告我们在肛周区域EGIST方面的经验并复习文献。一名70岁男性因肛门不适2年就诊于我院。盆腔磁共振成像扫描显示一个均匀、边界清晰的软组织密度肿块。患者接受了肿块切除术,病理检查证实该肿块为EGIST。肿瘤大小为4.3×3.2 cm,每50个高倍视野有1个核分裂象。肿瘤细胞免疫组化KIT和CD34呈阳性,但S-100和α-平滑肌肌动蛋白呈阴性。胃肠道无其他异常发现;经病理复查,该病例确诊为肛周EGIST。因此,EGIST应被视为肛周肿块的鉴别诊断之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5d65/9942734/10464b107307/kjco-16-2-138f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5d65/9942734/0d829cb43c0b/kjco-16-2-138f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5d65/9942734/22d8f335955a/kjco-16-2-138f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5d65/9942734/10464b107307/kjco-16-2-138f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5d65/9942734/0d829cb43c0b/kjco-16-2-138f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5d65/9942734/22d8f335955a/kjco-16-2-138f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5d65/9942734/10464b107307/kjco-16-2-138f3.jpg

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2
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引用本文的文献

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An update on gastrointestinal stromal tumors (GISTs) with a focus on extragastrointestinal stromal tumors (EGISTs).胃肠道间质瘤(GISTs)的最新进展,重点关注胃肠道外间质瘤(EGISTs)。
Gastroenterol Rep (Oxf). 2025 Jul 23;13:goaf068. doi: 10.1093/gastro/goaf068. eCollection 2025.

本文引用的文献

1
Extra-gastrointestinal stromal tumor arising in the lesser omentum with a platelet-derived growth factor receptor alpha (PDGFRA) mutation: a case report and literature review.发生于小网膜的胃外胃肠道间质瘤伴血小板衍生生长因子受体α(PDGFRA)突变:病例报告及文献复习。
World J Surg Oncol. 2020 Jul 23;18(1):183. doi: 10.1186/s12957-020-01961-1.
2
Retrospective analysis of extra-gastrointestinal stromal tumors.胃肠道外间质瘤的回顾性分析
World J Gastroenterol. 2015 Feb 14;21(6):1845-50. doi: 10.3748/wjg.v21.i6.1845.
3
Extragastrointestinal stromal tumors (EGIST): a series of case reports.
胃肠道外间质瘤(EGIST):一系列病例报告
Hepatogastroenterology. 2011 May-Jun;58(107-108):865-8.
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Gastrointestinal stromal tumors: pathology and prognosis at different sites.胃肠道间质瘤:不同部位的病理学与预后
Semin Diagn Pathol. 2006 May;23(2):70-83. doi: 10.1053/j.semdp.2006.09.001.
5
Gastrointestinal stromal tumours: a regular origin in the muscularis propria, but an extremely diverse gross presentation. A review of 200 cases to critically re-evaluate the concept of so-called extra-gastrointestinal stromal tumours.胃肠道间质瘤:起源于固有肌层,但大体表现极为多样。对200例病例的回顾,以严格重新评估所谓的胃肠道外间质瘤的概念。
Langenbecks Arch Surg. 2006 Aug;391(4):322-9. doi: 10.1007/s00423-005-0005-5. Epub 2006 Jan 10.
6
c-kit and PDGFRA mutations in extragastrointestinal stromal tumor (gastrointestinal stromal tumor of the soft tissue).胃肠道外间质瘤(软组织胃肠道间质瘤)中的c-kit和PDGFRA突变
Am J Surg Pathol. 2004 Apr;28(4):479-88. doi: 10.1097/00000478-200404000-00007.
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The histopathological differential diagnosis of gastrointestinal stromal tumours.胃肠道间质瘤的组织病理学鉴别诊断
J Clin Pathol. 2001 Feb;54(2):96-102. doi: 10.1136/jcp.54.2.96.
8
Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis.胃肠道间质瘤——定义、临床、组织学、免疫组化及分子遗传学特征与鉴别诊断
Virchows Arch. 2001 Jan;438(1):1-12. doi: 10.1007/s004280000338.
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Extragastrointestinal (soft tissue) stromal tumors: an analysis of 48 cases with emphasis on histologic predictors of outcome.胃肠道外(软组织)间质瘤:48例病例分析,重点关注预后的组织学预测指标。
Mod Pathol. 2000 May;13(5):577-85. doi: 10.1038/modpathol.3880099.