| 陈锦超,龚 侃,朱绍兴.VHL肾癌的自然病程研究进展[J].肿瘤学杂志,2018,24(7):729-733. |
| VHL肾癌的自然病程研究进展 |
| Research Progress of Nature History of Von Hippel-Lindau Renal Cell Carcinoma |
| 投稿时间：2017-08-06 |
| DOI：10.11735/j.issn.1671-170X.2018.07.B015 |
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| 中文关键词: VHL肾癌 自然病程 生长速率 转移 |
| 英文关键词:von Hippel-Lindau renal cell carcinoma natural history growth rate metastasis |
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| 中文摘要: |
| 摘 要： VHL肾癌是一种常见的遗传性肾癌，是VHL病患者的重要死亡原因。通过研究VHL肾癌的自然病程了解其生长、转移特点，可以指导临床监测和治疗。VHL肾癌生长速率存在较大变异，线性生长率为0~1.87cm/年，可能影响因素有肿瘤的生长阶段、初始肿瘤大小、基因突变类型等。VHL肾癌发生转移与肿瘤大小和生长速率呈正相关。直径小于3cm极少发生转移，因此目前指南建议3cm作为手术干预阈值。VHL肾癌和散发性肾癌的自然病程存在诸多差异，在治疗上存在明显区别，需要加以鉴别。 |
| 英文摘要: |
| Abstract：Von Hippel-Lindau (VHL) renal cell carcinoma is a common hereditary kidney cancer which is one of the most important causes of death in patients with VHL disease. To study the natural history of renal tumor associated with VHL disease and its characteristics of growth and metastasis，will facilitate the surveillance and treatment of this disease. The growth rate of VHL renal cell carcinoma varies greatly with range of 0 to 1.87cmper year. The factors related to the growth rate include stages of growth，primary tumor size and types of mutation. The metastasis is positively correlated with the size and growth rate of the tumors. Metastasis rarely occurs in the renal tumors with the diameter <3cm，so the guidelines suggest that 3cm should be the threshold for surgical intervention. There are many differences in nature history and treatment strategies between VHL renal cell carcinoma and sporadic kidney cancer，so it is necessary to distinguish these two types of renal tumors. |
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