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起源于空肠系膜的KIT(CD117)阳性巨大原发性恶性胃肠道外间质瘤(EGISTs):1例罕见病例报告

KIT (CD117) Positive Huge Primary Malignant Extra Gastrointestinal Stromal Tumors (EGISTs) Arising From Jejunal Mesentery: A Rare Case Report.

作者信息

Sajjanar Anita B, Katole Nilesh T, Vagha Sunita J

机构信息

Pathology, Datta Meghe Medical College, Datta Meghe Institute of Medical Sciences, Nagpur, IND.

Pharmacology and Therapeutics, Datta Meghe Medical College, Datta Meghe Institute of Medical Sciences, Nagpur, IND.

出版信息

Cureus. 2022 Dec 31;14(12):e33168. doi: 10.7759/cureus.33168. eCollection 2022 Dec.

DOI:10.7759/cureus.33168
PMID:36742272
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9891314/
Abstract

Tumors arising outside gastrointestinal systems are known as extra gastrointestinal stromal tumors (EGISTs). Outside gastrointestinal sites include the mesentery, omentum, peritoneum, pancreas, and liver. Our case highlights a rare occurrence of an EGIST in jejunal mesentery in a 45-year-old male with an asymptomatic large abdominal growth and weight loss. A contrast-enhanced multi-dimensional computed tomography scan showed a large heterogeneous mass in the left hypochondrium. lumbar, and paraumbilical regions. Later, the patient underwent surgical resection of the tumor along with the involved jejunal segment and small tumor masses in the mesentery. Histopathological examination reported a malignant EGIST of mesentery and invasion into the jejunum, further confirmed by immunohistochemistry (IHC) markers like CD117 and smooth muscle actin with a high proliferative index (Ki67). One should be aware that these are different from other malignancies arising from the mesentery. Their cell of origin is different and needs a specific type of treatment. The clinical history, radiological findings, histopathology, and IHC help in diagnosing especially when they are arising from unusual areas like jejunal mesentery. Surgical intervention and chemotherapy are mainstay treatments.

摘要

起源于胃肠道系统之外的肿瘤被称为胃肠道外间质瘤(EGIST)。胃肠道外部位包括肠系膜、网膜、腹膜、胰腺和肝脏。我们的病例突出显示了一名45岁男性空肠系膜中罕见的EGIST发生情况,该患者有一个无症状的腹部巨大肿物并伴有体重减轻。增强型多维计算机断层扫描显示左季肋部、腰部和脐旁区域有一个巨大的异质性肿块。后来,患者接受了肿瘤连同受累空肠段以及肠系膜中小肿瘤块的手术切除。组织病理学检查报告为肠系膜恶性EGIST并侵犯空肠,通过免疫组织化学(IHC)标记物如CD117和平滑肌肌动蛋白进一步证实,增殖指数(Ki67)较高。应注意这些肿瘤与起源于肠系膜的其他恶性肿瘤不同。它们的起源细胞不同,需要特定类型的治疗。临床病史、放射学表现、组织病理学和免疫组织化学有助于诊断,尤其是当它们起源于空肠系膜等不寻常区域时。手术干预和化疗是主要治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ff0/9891314/11b60a9d6286/cureus-0014-00000033168-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ff0/9891314/a4b5666294aa/cureus-0014-00000033168-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ff0/9891314/7206bf0dbf67/cureus-0014-00000033168-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ff0/9891314/11b60a9d6286/cureus-0014-00000033168-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ff0/9891314/a4b5666294aa/cureus-0014-00000033168-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ff0/9891314/7206bf0dbf67/cureus-0014-00000033168-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ff0/9891314/11b60a9d6286/cureus-0014-00000033168-i03.jpg

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本文引用的文献

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J Pers Med. 2021 Jul 22;11(8):694. doi: 10.3390/jpm11080694.
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Extragastrointestinal stromal tumour (EGIST) presented as a mesenteric and retroperitoneal mass.胃肠道外间质瘤(EGIST)表现为肠系膜和腹膜后肿块。
BMJ Case Rep. 2019 Dec 2;12(12):e232481. doi: 10.1136/bcr-2019-232481.
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Extragastrointestinal Stromal Tumor Presenting as Omental Cyst.表现为网膜囊肿的胃肠道外间质瘤
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