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右半结肠侵袭性纤维瘤病酷似胃肠道间质瘤:一例报告

Aggressive fibromatosis of the right colon mimicking a gastrointestinal stromal tumour: a case report.

作者信息

Mitrovic Jovanovic Milica, Djuric-Stefanovic Aleksandra, Velickovic Dejan, Keramatollah Ebrahimi, Micev Marijan, Jankovic Aleksandra, Milosevic Stefan, D Kovac Jelena

机构信息

Centre for Radiology and MRI, University Clinical Centre of Serbia, Belgrade, Serbia.

Faculty of Medicine, University of Belgrade, Belgrade, Serbia.

出版信息

J Int Med Res. 2021 Mar;49(3):300060521994927. doi: 10.1177/0300060521994927.

DOI:10.1177/0300060521994927
PMID:33752489
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7995451/
Abstract

Aggressive fibromatosis is a rare type of intra-abdominal desmoid tumour that usually involves the small bowel mesentery. It is a locally-invasive lesion, with a high rate of recurrence, but without metastatic potential. Aggressive fibromatosis is seen more often in young female patients. This case report presents the radiological, intraoperative and histopathological findings from a 37-year-old female patient that presented with epigastric pain and a palpable mass in the right hemiabdomen. Histological and immunohistochemical examinations of the resected tumour, including positive staining for beta-catenin, confirmed a postoperative diagnosis of desmoid type fibromatosis. This specific case showed that desmoid type fibromatosis of the colon can mimic gastrointestinal stromal tumours (GIST) based on its clinical presentation, computed tomography and magnetic resonance imaging findings. Differential diagnosis between desmoid type fibromatosis and GIST is clinically very important due to the different treatments and follow-up protocols that are implemented for these lesions.

摘要

侵袭性纤维瘤病是一种罕见的腹腔内硬纤维瘤,通常累及小肠系膜。它是一种局部侵袭性病变,复发率高,但无转移潜能。侵袭性纤维瘤病在年轻女性患者中更为常见。本病例报告展示了一名37岁女性患者的影像学、术中及组织病理学检查结果,该患者表现为上腹部疼痛且右半腹可触及肿块。对切除肿瘤进行的组织学和免疫组化检查,包括β-连环蛋白阳性染色,确诊为术后硬纤维瘤型纤维瘤病。这个特殊病例表明,结肠硬纤维瘤型纤维瘤病基于其临床表现、计算机断层扫描和磁共振成像结果可酷似胃肠道间质瘤(GIST)。由于对这些病变实施的治疗和随访方案不同,硬纤维瘤型纤维瘤病与GIST之间的鉴别诊断在临床上非常重要。

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World J Gastrointest Oncol. 2024 Aug 15;16(8):3716-3722. doi: 10.4251/wjgo.v16.i8.3716.
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An unusual case of mesenteric fibromatosis.肠系膜纤维瘤病一例罕见病例。
Kaohsiung J Med Sci. 2024 May;40(5):511-512. doi: 10.1002/kjm2.12817. Epub 2024 Mar 1.
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Large desmoid tumour of the small bowel mesentery.小肠系膜巨大硬纤维瘤。

本文引用的文献

1
Ruptured desmoid tumor imitating acute appendicitis - a rare reason for an emergency surgery.模仿急性阑尾炎的韧带样纤维瘤破裂——急诊手术的罕见原因。
BMC Surg. 2019 Dec 16;19(1):194. doi: 10.1186/s12893-019-0662-x.
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Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study.帕唑帕尼或甲氨蝶呤-长春碱联合化疗治疗进展性硬纤维瘤成人患者(DESMOPAZ):一项非比较、随机、开放标签、多中心、2 期研究。
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An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG).
关于散发性硬纤维瘤样纤维瘤病(desmoid-type fibromatosis)治疗的最新进展:肉瘤患者欧洲网络(Sarcoma PAtients EuroNet,SPAEN)和欧洲癌症研究与治疗组织(European Organization for Research and Treatment of Cancer,EORTC)/软组织和骨肿瘤研究组(Soft Tissue and Bone Sarcoma Group,STBSG)之间的欧洲共识倡议。
Ann Oncol. 2017 Oct 1;28(10):2399-2408. doi: 10.1093/annonc/mdx323.
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Treatment and follow-up strategies in desmoid tumours: a practice guideline.纤维瘤病的治疗和随访策略:实践指南。
Curr Oncol. 2014 Aug;21(4):e642-9. doi: 10.3747/co.21.2112.
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Desmoid infilterating ileum, a rare complication.硬纤维瘤浸润回肠,一种罕见的并发症。
Indian J Surg. 2013 Jun;75(Suppl 1):192-4. doi: 10.1007/s12262-012-0564-y. Epub 2012 Jun 23.
6
Mesenteric fibromatosis with intestinal involvement mimicking a gastrointestinal stromal tumour.肠系膜纤维瘤病伴肠受累,表现类似胃肠道间质瘤。
Radiol Oncol. 2011 Mar;45(1):59-63. doi: 10.2478/v10019-010-0051-7. Epub 2010 Nov 25.
7
Desmoid tumour (aggressive fibromatosis) of the colon mimics malignancy on dual time-point 18F-FDG PET/CT imaging.结肠侵袭性纤维瘤病(硬纤维瘤)在双时相 18F-FDG PET/CT 成像上模拟恶性肿瘤。
Br J Radiol. 2012 Feb;85(1010):e37-40. doi: 10.1259/bjr/43870228.
8
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