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Natural history of renal tumours in von Hippel-Lindau disease: a large retrospective study of Chinese patients
  1. Xiang Peng1,2,3,4,
  2. Jinchao Chen5,
  3. Jiangyi Wang6,7,
  4. Shuanghe Peng1,3,4,
  5. Shengjie Liu1,3,4,
  6. Kaifang Ma1,3,4,
  7. Jingcheng Zhou1,3,4,
  8. Baoan Hong1,3,4,
  9. Bowen Zhou1,3,4,
  10. Jiufeng Zhang1,3,4,
  11. Lin Cai1,3,4,
  12. Kan Gong1,3,4
  1. 1 Department of Urology, Peking University First Hospital, Beijing, China
  2. 2 Department of Urology, The Second Affiliated Hospital of NanChang University, Jiangxi, China
  3. 3 Institute of Urology, Peking University, Beijing, China
  4. 4 National Urological Cancer Center, Beijing, China
  5. 5 Department of Urology, Zhejiang Cancer Hospital, Zhejiang, China
  6. 6 Department of Urology, Fudan University Shanghai Cancer Center, Shanghai, China
  7. 7 Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
  1. Correspondence to Dr Lin Cai and Professor Kan Gong, Department of Urology, Peking University First Hospital Institute of Urology, Peking University National Urological Cancer Center, Beijing, 100034, China; drcailin{at}, gongkan_pku{at}


Background Historically, renal cell carcinoma (RCC) is one of the main causes of death in von Hippel-Lindau (VHL) disease. However, the natural history of VHL-related RCC has not been thoroughly elucidated to date. This report described the natural history of VHL-related RCC in a large Chinese VHL cohort and might be helpful in the surveillance and treatment of VHL disease.

Methods In this retrospective study, we included 196 renal tumours from 150 patients with VHL disease. Statistical analysis was used to evaluate the influence of age of onset, sex, family history, unilateral or bilateral tumour, VHL disease type, mutation type, mutation location, and tumour size on tumour growth, metastasis and survival in patients with VHL disease.

Results The mean age of onset was 38.8 years, and the mean initial tumour size was 3.1 cm. The mean linear growth rate was 0.49 cm/year. Patients experienced faster tumour growth when they had later age of onset, larger initial tumour size, missense mutation, mutations locating in exon 3, and when they were not affected by cerebral or retinal haemangioblastomas. Tumours larger than 4 cm grew faster than those smaller than 4 cm. Bilateral tumours, large initial tumours, fast tumour growth and metastasis were risk factors for poor prognosis in VHL-related RCC.

Conclusion This large study demonstrated that age of onset, initial tumour size, concomitant tumours, mutation type and mutation location had an effect on growth rate in VHL-related RCC. Active surveillance may be safe for patients with tumour size less than 4 cm, which is helpful in clinical decision-making.

  • vhl disease
  • renal cell carcinoma
  • natural history
  • growth rate
  • prognosis

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  • XP, JC and JW contributed equally.

  • Contributors KG and LC were responsible for the concept and design of the study. XP, JC and JW contributed equally to this work. XP, JC and JW dealt with the clinical data. XP, SP and SL performed the statistical work. JinZ, KM and BH drafted the manuscript. JiuZ and BZ provided the figures and tables. All authors revised the manuscript.

  • Funding This study was supported by the National Natural Science Foundation of China (Grant Number: 81572506), the Special Health Development Research Project of Capital (Grant Number: 2016-2-4074) and the Beijing Municipal Science and Technology Commission (Grant Number: Z151100003915126).

  • Competing interests None declared.

  • Ethics approval The project was approved by the Medical Ethics Committee of Peking University First Hospital. Informed consent was obtained from patients or legal guardians.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Correction notice This article has been corrected since it was published online first. The affiliations and the equal contribution statement have been corrected.