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酷似胃肠道间质瘤的腹腔内小肠硬纤维瘤

Intra-abdominal small intestinal desmoid tumour mimicking GIST.

作者信息

Eswaravaka Sai Krishna, Deshpande Swanit Hemant, Chiranjeev Roshan, Pandya Jayashri Sanjay

机构信息

Department of General Surgery, Topiwala National Medical College, Mumbai, Maharashtra, India.

General Surgery, BYL Nair Charitable Hospital, Mumbai, Maharashtra, India.

出版信息

BMJ Case Rep. 2021 Feb 22;14(2):e237032. doi: 10.1136/bcr-2020-237032.

Abstract

Desmoid tumours, also known as aggressive fibromatosis, are fibromuscular neoplasms that arise from mesenchymal cell lines. Desmoid tumours are usually benign and are locally aggressive tumours. We report a case of a 31-year-old man presenting with abdominal mass associated with dyspepsia and early satiety. CT scan demonstrated a large heterogeneous mass adherent to or arising from the jejunum. The patient underwent a successful elective exploratory laparotomy with resection of the tumour arising from the wall of the ileum with a 10 cm margin. The patient had an uneventful recovery and no recurrence at 6-month follow-up. Pathology report and immunohistochemistry analysis revealed the mass to be a primary desmoid tumour of the small bowel, as the tumour was negative for c-kit and Discovered on GIST 1 (DOG-1) and positive for beta-catenin and smooth muscle actin.

摘要

硬纤维瘤,也称为侵袭性纤维瘤病,是起源于间充质细胞系的纤维肌肉肿瘤。硬纤维瘤通常为良性,但具有局部侵袭性。我们报告一例31岁男性患者,表现为腹部肿块,伴有消化不良和早饱感。CT扫描显示一个大的不均匀肿块,附着于空肠或起源于空肠。患者接受了成功的选择性剖腹探查术,切除了起源于回肠壁的肿瘤,切缘为10厘米。患者恢复顺利,6个月随访时无复发。病理报告和免疫组化分析显示该肿块为小肠原发性硬纤维瘤,因为该肿瘤c-kit和GIST 1(DOG-1)检测为阴性,β-连环蛋白和平滑肌肌动蛋白检测为阳性。

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引用本文的文献

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Distal Duodenal Stricture Secondary to Mesenteric Fibromatosis (Intra-Abdominal Desmoid Tumor) of the Jejunum.
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