Journal of Cancer Research and Clinical Oncology

, Volume 140, Issue 7, pp 1171–1177 | Cite as

Renal tumor size is an independent prognostic factor for overall survival in von Hippel–Lindau disease

  • Taekmin Kwon
  • In Gab Jeong
  • Sahyun Pak
  • Dalsan You
  • Cheryn Song
  • Jun Hyuk Hong
  • Hanjong Ahn
  • Choung-Soo KimEmail author
Original Article – Clinical Oncology



To evaluate the effect of renal cell carcinoma (RCC) on survival in von Hippel–Lindau (VHL) disease and to assess the relationship between tumor size and survival.

Materials and methods

In this retrospective cohort study, the medical records of 72 patients who presented with VHL disease between 1994 and 2012 were reviewed. Clinical VHL-related characteristics were analyzed, and the prognostic value of renal tumor size for overall survival was assessed by using Cox regression models.


Of the 72 VHL patients, 42 (58.3 %) and 30 (41.7 %) were male and female, respectively. The mean age was 37.9 years, and the median follow-up period was 61.5 months. In terms of VHL-related manifestations, 40 (55.6 %) had RCC, 46 (63.8 %) had hemangioblastoma in the cerebellum, 10 (13.9 %) had hemangioblastoma in the spinal cord, 34 (47.2 %) had a pancreatic mass, 18 (25.0 %) had pheochromocytoma, and 14 (19.4 %) had retinal capillary hemangioma. RCC was a major cause of mortality: Of the 11 patients who died, nine (12.5 %) died due to RCC progression. The 5-year overall survival rate was 85.6 % for all patients, 96.9 % for patients without RCC, 83.6 % for patients with RCC < 3 cm, and 75.8 % for patients with RCC ≥ 3 cm. Multivariable analysis showed that RCC ≥ 3 cm was an independent predictor of overall survival (HR 9.87, 95 % CI 1.17–83.00, p = 0.035) along with age (HR 1.05, 95 % CI 1.01–1.10, p = 0.027).


Renal tumor size was an independent prognostic factor for overall survival in VHL disease. This observation will be helpful for planning RCC treatment in VHL disease.


Von Hippel–Lindau disease Carcinoma Renal cell Prognosis 



The authors have no conflict of interests or financial disclosures.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Taekmin Kwon
    • 1
  • In Gab Jeong
    • 1
  • Sahyun Pak
    • 1
  • Dalsan You
    • 1
  • Cheryn Song
    • 1
  • Jun Hyuk Hong
    • 1
  • Hanjong Ahn
    • 1
  • Choung-Soo Kim
    • 1
    Email author
  1. 1.Department of Urology, Asan Medical CenterUniversity of Ulsan College of MedicineSeoulKorea

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