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一名儿科患者的硬纤维瘤病罕见病例:手术治疗及结果

A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and Outcomes.

作者信息

Yeap Boon Tat, Nasir Ikhwan, Koo Thai Hau, Paul Aaron Gerarde, Zakaria Mohd Hazeman

机构信息

Department of Anaesthesiology and Intensive Care, Hospital Universiti Malaysia Sabah, Kota Kinabalu, Sabah, Malaysia.

Faculty of Medicine and Health Sciences, Universiti Malaysia Sabah, Kota Kinabalu, Sabah, Malaysia.

出版信息

Radiol Case Rep. 2025 Mar 8;20(5):2384-2387. doi: 10.1016/j.radcr.2025.01.083. eCollection 2025 May.

DOI:10.1016/j.radcr.2025.01.083
PMID:40129797
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11930522/
Abstract

Desmoid fibromatosis (DF) is a rare, locally aggressive tumor arising from the abdominal fascia or musculoaponeurosis, commonly affecting individuals between 15 and 60 years of age. We present a case of a 13-year-old boy with a 9-month history of progressive swelling in the left posterior thigh. Magnetic resonance imaging (MRI) revealed a soft tissue tumor encasing the sciatic nerve. A biopsy confirmed the diagnosis of DF, showing spindle cells arranged in fascicles. The patient underwent wide local excision of the tumor with preservation of the sciatic nerve. Postoperative recovery was smooth, and 1-year follow-up MRI showed no recurrence. Surgical excision remains the primary treatment, especially in symptomatic patients, although recurrence is common even with negative margins. This case underscores the importance of regular follow-up for DF and a multidisciplinary approach to optimize management and surveillance.

摘要

韧带样纤维瘤病(DF)是一种罕见的、局部侵袭性肿瘤,起源于腹部筋膜或肌腱膜,常见于15至60岁的个体。我们报告一例13岁男孩,左大腿后部进行性肿胀9个月。磁共振成像(MRI)显示一个包裹坐骨神经的软组织肿瘤。活检确诊为DF,可见梭形细胞呈束状排列。患者接受了肿瘤广泛局部切除并保留了坐骨神经。术后恢复顺利,1年随访MRI显示无复发。手术切除仍然是主要治疗方法,尤其是对有症状的患者,尽管切缘阴性时复发也很常见。该病例强调了DF定期随访以及采用多学科方法优化管理和监测的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4957/11930522/bebdb2e3b02a/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4957/11930522/0557e5baa616/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4957/11930522/064b5d73d3fe/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4957/11930522/27c51e311633/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4957/11930522/bebdb2e3b02a/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4957/11930522/0557e5baa616/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4957/11930522/064b5d73d3fe/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4957/11930522/27c51e311633/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4957/11930522/bebdb2e3b02a/gr4.jpg

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