| 中文关键词: 四肢 韧带样纤维瘤病 治疗 |
| 英文关键词:limb desmoid fibromatosis treatment |
| 基金项目:广西卫计委自筹课题项目（Z2016313）；广西医科大学青年基金项目（GXMUYSF201510） |
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| 中文摘要: |
| 摘 要：四肢韧带样纤维瘤病是一种罕见的缺乏转移能力、具有局部侵袭潜能且复发性高的间质肿瘤。其临床病程多变，但罕见恶变，因侵袭性生长，局部易复发。目前治疗以观察等待为主，辅以局部治疗（手术、放疗等）与系统治疗（激素类、非甾体类抗炎药物治疗等），并密切随访监测其临床变化与影像学改变，如肿瘤进展，可根据病情选择个体化、综合性的临床干预方案，从而获得良好的远期无病生存率。 |
| 英文摘要: |
| Abstract：Limb desmoid fibromatosis is a rare，locally aggressive，mesenchymal neoplasm that is associated with high rates of local recurrence but lacks the potential to metastasis. Its clinical course is unpredictable，but is rare to be malignant. The active wait-and-see approach is given as the first-line management，supplemented with local treatment (surgery or radiotherapy) and systematic treatment (steroid hormones or nonsteroidal anti-inflammatory drugs). The patients should be closely monitored for clinical and imaging changes. Once the tumor progresses，the comprehensive clinical intervention should be given according to the individual conditions to obtain long-term disease-free survival. |
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