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具有挑战性的诊断难题:伪装成穿孔性腹膜炎的肠系膜硬纤维瘤。

Challenging Diagnostic Dilemma: Mesenteric Desmoid Tumor Masquerading as Perforated Peritonitis.

作者信息

Das Siddharth Sankar, Navlani Sahil, Alawa Arfan, Abbas Ferial M, Raman Lakshmiah G, Mestha Akshata

机构信息

General Surgery, Dubai Hospital, Dubai, ARE.

Medical School, Dubai Academic Health Corporation, Dubai, ARE.

出版信息

Cureus. 2023 Aug 4;15(8):e42946. doi: 10.7759/cureus.42946. eCollection 2023 Aug.

DOI:10.7759/cureus.42946
PMID:37667699
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10475247/
Abstract

Desmoid fibromatosis is a rare benign neoplasm of the soft tissue. Primary desmoid neoplasms rarely occur in the small bowel and are primarily found in patients with a previous abdominal surgery or irradiation history. They are challenging to diagnose at the time of presentation due to a lower incidence and their non-specific presentation making it difficult to distinguish from other intra-abdominal neoplasms, such as gastrointestinal stromal tumors (GISTs), which may present with similar symptoms. We like to present a case of a 34-year-old male with a four-day history of abdominal pain with worsening severity and one episode of non-bloody vomiting. Physical examination was significant for generalized abdominal tenderness with positive rebound and board-like rigidity. A computed tomography (CT) scan of the abdomen showed the presence of a lower abdominal mass of unknown etiology with free air foci and free intraperitoneal fluid either due to rupture of the suspicious mass or secondary to infection by an air-producing organism. The patient was immediately taken for emergency surgery, the tumor was resected successfully, and a specimen collected was sent for histopathology, which came out to be a desmoid tumor. We aim to highlight the importance of keeping a broad differential diagnosis in a patient with acute abdomen and symptoms of peritonitis.

摘要

硬纤维瘤病是一种罕见的软组织良性肿瘤。原发性硬纤维瘤很少发生于小肠,主要见于既往有腹部手术或放疗史的患者。由于其发病率较低且临床表现不具特异性,难以与其他腹腔内肿瘤(如胃肠道间质瘤(GISTs))相鉴别,因此在疾病呈现时诊断具有挑战性,这些肿瘤可能表现出相似的症状。我们在此呈现一例34岁男性患者,有四天的腹痛病史,疼痛程度逐渐加重,并有一次非血性呕吐。体格检查显示全腹压痛明显,有反跳痛及板状腹。腹部计算机断层扫描(CT)显示下腹部有一病因不明的肿块,伴有游离气体灶及腹腔内游离液体,这可能是由于可疑肿块破裂或产气菌感染所致。患者立即接受急诊手术,肿瘤被成功切除,所采集的标本送去做组织病理学检查,结果为硬纤维瘤。我们旨在强调对有急腹症和腹膜炎症状的患者进行广泛鉴别诊断的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/f189f31a2da6/cureus-0015-00000042946-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/ca24c77294f7/cureus-0015-00000042946-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/41d8709d9e86/cureus-0015-00000042946-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/e4502472fbbe/cureus-0015-00000042946-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/3e3542c3a2c5/cureus-0015-00000042946-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/f189f31a2da6/cureus-0015-00000042946-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/ca24c77294f7/cureus-0015-00000042946-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/41d8709d9e86/cureus-0015-00000042946-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/e4502472fbbe/cureus-0015-00000042946-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/3e3542c3a2c5/cureus-0015-00000042946-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e06/10475247/f189f31a2da6/cureus-0015-00000042946-i05.jpg

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本文引用的文献

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Desmoid Tumors Arising on the Mesenteric Surgical Scar of Abdominal Sarcomas.腹部肉瘤肠系膜手术瘢痕处发生的硬纤维瘤
Cureus. 2022 Jan 30;14(1):e21727. doi: 10.7759/cureus.21727. eCollection 2022 Jan.
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Desmoid Fibromatosis: Management in an Era of Increasing Options.韧带样纤维瘤病:治疗选择日益增多时代的管理。
Curr Oncol Rep. 2021 Mar 14;23(4):41. doi: 10.1007/s11912-021-01026-w.
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Eur J Cancer. 2020 Mar;127:96-107. doi: 10.1016/j.ejca.2019.11.013. Epub 2020 Jan 28.
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Desmoid Tumor as an Initial Presentation of Familial Adenomatous Polyposis: A Review of the Literature.韧带样瘤作为家族性腺瘤性息肉病的首发表现:文献综述
Cureus. 2018 Mar 9;10(3):e2297. doi: 10.7759/cureus.2297.
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Pazopanib therapy for desmoid tumors in adolescent and young adult patients.帕唑帕尼治疗青少年和年轻成年患者的硬纤维瘤。
Pediatr Blood Cancer. 2018 Jun;65(6):e26968. doi: 10.1002/pbc.26968. Epub 2018 Jan 31.
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