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一例罕见的穿孔性巨大肠系膜纤维瘤病导致急性腹膜炎。

A rare case of perforated giant mesenteric fibromatosis causing acute peritonitis.

作者信息

Ibrahim Mohanad, Toffaha Ali, Khawar Mahwish, Abdul-Hafez Hamza A, Kurer Mohamed

机构信息

Department of General Surgery, Hamad Medical Corporation, Doha, Qatar.

Colorectal Surgery Unit, Hamad Medical Corporation, Doha, Qatar.

出版信息

J Surg Case Rep. 2025 Jul 3;2025(7):rjaf459. doi: 10.1093/jscr/rjaf459. eCollection 2025 Jul.

DOI:10.1093/jscr/rjaf459
PMID:40620786
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12225994/
Abstract

Desmoid tumors (DT) are rare benign tumors originating from myofibroblasts in the mesentery. Although benign, DTs exhibit local invasion and recurrence and may present sporadically or with familial adenomatous polyposis (FAP) and Gardner's syndrome. The clinical presentation ranges from asymptomatic abdominal masses to severe complications such as bowel obstruction and perforation. This case report highlights a 46-year-old male with acute diffuse peritonitis due to perforation of a mesenteric fibromatosis (MF). Imaging revealed a large mesenteric mass causing bowel perforation, necessitating an urgent exploratory laparotomy. Surgical intervention entailed resection of the affected small bowel and mesentery with temporary stoma formation. Histopathology confirmed MF with negative margins, and the patient was discharged in stable condition. This case highlights the importance of a multidisciplinary approach in managing such complex cases, the challenges in diagnosing and treating intra-abdominal DTs, and the role of surgical resection with careful postoperative monitoring despite recurrence concerns.

摘要

韧带样型纤维瘤病(DT)是起源于肠系膜肌成纤维细胞的罕见良性肿瘤。尽管是良性肿瘤,但DT具有局部侵袭性和复发性,可散发出现或与家族性腺瘤性息肉病(FAP)及加德纳综合征相关。临床表现从无症状腹部肿块到肠梗阻和穿孔等严重并发症不等。本病例报告重点介绍了一名46岁男性,因肠系膜纤维瘤病(MF)穿孔导致急性弥漫性腹膜炎。影像学检查发现一个导致肠穿孔的巨大肠系膜肿块,需要紧急进行剖腹探查术。手术干预包括切除受影响的小肠和肠系膜并临时造口。组织病理学证实为MF,切缘阴性,患者出院时病情稳定。本病例强调了多学科方法在处理此类复杂病例中的重要性、诊断和治疗腹腔内DT的挑战,以及尽管存在复发担忧但手术切除并进行仔细术后监测的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0771/12225994/55e441604b10/rjaf459f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0771/12225994/4e88a723a445/rjaf459f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0771/12225994/55e441604b10/rjaf459f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0771/12225994/4e88a723a445/rjaf459f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0771/12225994/55e441604b10/rjaf459f2.jpg

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本文引用的文献

1
Desmoid Tumors: A Comprehensive Review.韧带样纤维瘤:全面综述。
Adv Ther. 2023 Sep;40(9):3697-3722. doi: 10.1007/s12325-023-02592-0. Epub 2023 Jul 12.
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Case report: Desmoid fibromatosis diagnosed in a 27-year-old male after being mistaken for a gastrointestinal stromal tumour.病例报告:一名27岁男性被误诊为胃肠道间质瘤后,最终确诊为韧带样纤维瘤病。
Front Med (Lausanne). 2022 Nov 10;9:998473. doi: 10.3389/fmed.2022.998473. eCollection 2022.
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Gastrointestinal Complications of Intra-Abdominal Desmoid Tumors: A Case Report and Review of the Literature.
腹腔内硬纤维瘤的胃肠道并发症:一例报告及文献复习
Case Rep Oncol. 2022 May 12;15(2):515-521. doi: 10.1159/000522441. eCollection 2022 May-Aug.
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Huge mesenteric fibromatosis presenting with intestinal perforation and acute diffuse peritonitis: a case report.巨大肠系膜纤维瘤病伴肠穿孔及急性弥漫性腹膜炎:一例报告
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Locally Aggressive Connective Tissue Tumors.局部侵袭性结缔组织肿瘤。
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Role of Imaging in Management of Desmoid-type Fibromatosis: A Primer for Radiologists.影像学在韧带样型纤维瘤病管理中的作用:放射科医生入门指南
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Management of desmoid tumours: A nationwide survey of labelled reference centre networks in France.硬纤维瘤的管理:法国标记参考中心网络的全国性调查。
Eur J Cancer. 2016 May;58:90-6. doi: 10.1016/j.ejca.2016.02.008. Epub 2016 Mar 11.
9
Mesenteric fibromatosis (desmoid tumour) - a rare case report.肠系膜纤维瘤病(硬纤维瘤)——一例罕见病例报告。
J Clin Diagn Res. 2014 Nov;8(11):ND01-2. doi: 10.7860/JCDR/2014/8520.5098. Epub 2014 Nov 20.
10
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.