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原发性腹腔内硬纤维瘤病合并家族性腺瘤性息肉病：一例报告。

Primary intra‑abdominal desmoid fibromatosis associated with familial adenomatous polyposis: A case report.

作者信息

Zhang Lei, Zheng Yaotun, Yu Xiaoqian, Yu Kang, Zhu Shengjie

机构信息

Department of Hepatobiliary Surgery, Weihai Central Hospital, Weihai, Shandong 264200, P.R. China.

Department of Anesthesia, Weihai Central Hospital, Weihai, Shandong 264200, P.R. China.

出版信息

Exp Ther Med. 2023 May 10;26(1):308. doi: 10.3892/etm.2023.12007. eCollection 2023 Jul.

DOI:10.3892/etm.2023.12007
PMID:37273755
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10236137/
Abstract

Desmoid fibromatosis (DF) is a clonal proliferative disorder of myofibroblasts, which arises, with a low incidence, in soft tissue, including within the abdomen. The incidence of DF is associated with familial adenomatous polyposis (FAP), and is more common following FAP surgery. It is rare for a patient to make his/her first visit to hospital due to DF symptoms associated with FAP. In the present report, a case of mesenteric DF associated with FAP is described. This case also had incomplete intestinal obstruction due to DF. By summarizing previous studies examining DF and FAP treatment, combined with the disease characteristics of this patient, the clinical treatment strategy for DF associated with FAP was explored.

摘要

韧带样型纤维瘤病（DF）是一种肌成纤维细胞的克隆性增殖性疾病，发病率较低，发生于软组织，包括腹部。DF的发病率与家族性腺瘤性息肉病（FAP）相关，在FAP手术后更为常见。因与FAP相关的DF症状首次就诊的患者较为罕见。在本报告中，描述了一例与FAP相关的肠系膜DF病例。该病例还因DF导致不完全性肠梗阻。通过总结以往关于DF和FAP治疗的研究，并结合该患者的疾病特征，探讨了与FAP相关的DF的临床治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/8049e18d76f0/etm-26-01-12007-g04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/dc1089dcdd77/etm-26-01-12007-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/14b07d64db85/etm-26-01-12007-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/9c619e32fbf9/etm-26-01-12007-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/f985dd337aa3/etm-26-01-12007-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/8049e18d76f0/etm-26-01-12007-g04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/dc1089dcdd77/etm-26-01-12007-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/14b07d64db85/etm-26-01-12007-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/9c619e32fbf9/etm-26-01-12007-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/f985dd337aa3/etm-26-01-12007-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23ef/10236137/8049e18d76f0/etm-26-01-12007-g04.jpg

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Hereditary Cancer Syndromes-A Primer on Diagnosis and Management, Part 2: Gastrointestinal Cancer Syndromes.
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Sorafenib for Advanced and Refractory Desmoid Tumors.索拉非尼治疗晚期和难治性硬纤维瘤。
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