title: Renal tumor size is an independent prognostic factor for overall survival in von Hippel-Lindau disease.
abstract: OBJECTIVE
  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.
  
  
  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.
  
  
  RESULTS
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Urology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pungnap 2 dong, Songpa-gu, Seoul, 138-736, Korea.
authors.forename: Taekmin
authors.initials: T
authors.lastname: Kwon
authors.forename: In Gab
authors.initials: IG
authors.lastname: Jeong
authors.forename: Sahyun
authors.initials: S
authors.lastname: Pak
authors.forename: Dalsan
authors.initials: D
authors.lastname: You
authors.forename: Cheryn
authors.initials: C
authors.lastname: Song
authors.forename: Jun Hyuk
authors.initials: JH
authors.lastname: Hong
authors.forename: Hanjong
authors.initials: H
authors.lastname: Ahn
authors.forename: Choung-Soo
authors.initials: CS
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002292:Carcinoma, Renal Cell / Q000150:complications / Q000473:pathology*; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis; D047368:Tumor Burden; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000401:mortality*