结缔组织样肿瘤的治疗困境:一例报告

Q4 Medicine
F. Y. Lee, A. Jagwani, Mohamad Fairuz Mohamad Sharin, Andee Dzulkarnain Zakaria, F. Hayati, I. Sagap
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引用次数: 0

摘要

筛状瘤是罕见的良性肿瘤。肿瘤可能是偶发性发展的,也可能与Gardner综合征中的家族性腺瘤性息肉病(FAP)有关。这篇文章重点介绍了一位患有腹内硬纤维瘤的年轻女士,表现为急腹症,我们讨论了我们的治疗策略。一位患有已知FAP的24岁女性接受了全结肠切除术、回肠袋-肛门吻合术和甲状腺全切除术,后来因症状性硬纤维瘤并发急腹症。腹部的计算机断层扫描显示腹腔内有一个巨大的不均匀肿块,伴有小肠移位,并经磁共振成像证实。进行了剖腹探查和肿瘤的整体切除,并进行了末端回肠造口术。腹部硬纤维瘤通常表现为无痛的缓慢生长的肿块,然而,在严重的情况下,它会导致全结肠切除术后的肠缺血、肠梗阻或回肠-oanal吻合功能恶化。在特定的临床情况下,即并发症(闭塞、穿孔或出血)或重大美容问题中,手术是预先指示的。Desmoid肿瘤虽然是良性的,但由于其压迫性,其治疗具有挑战性。作为一个关键点,对于有FAP病史并伴有结肠外表现的患者,应怀疑并密切随访硬纤维瘤的诊断。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The dilemma in management of desmoid tumour: A case report
Desmoid tumours are rare benign tumours. The tumours may develop sporadically or they may be linked to familial adenomatous polyposis (FAP) in Gardner’s syndrome.This article highlights a young lady with an intra-abdominal desmoid tumour that manifested as an acute abdomen and we discuss our management strategy.A 24-year-old lady with a known FAP who had undergone a panproctocolectomy with an ileal pouch-anal anastomosis and total thyroidectomy, later was complicated with acute abdomen for symptomatic desmoid tumour. Computed tomography of the abdomen showed a large intraperitoneal heterogenous mass with small bowel displacement and was confirmed by magnetic resonance imaging. An exploratory laparotomy and en-bloc resection of the tumour with an end ileostomy were performed.Intra-abdominal desmoid tumours usually present as a painless slow-growing mass, however, in severe form, it can cause bowel ischemia, intestinal obstruction, or deterioration of function in the ileoanal anastomosis, among post total colectomy. Surgery is indicated upfront in specific clinical scenarios namely complications (occlusion, perforation, or bleeding) or major cosmetic issues.Desmoid tumour, despite being benign, is challenging to manage due to its compressing nature. As a key point, the diagnosis of a desmoid tumour should be suspected and followed up closely in patients with a previous history of FAP in combination with extracolonic manifestation.
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来源期刊
Polish Annals of Medicine
Polish Annals of Medicine Medicine-Medicine (all)
CiteScore
0.40
自引率
0.00%
发文量
28
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