title: The impact of family history on pathological and clinical outcomes in non-syndromic clear cell renal cell carcinoma.
abstract: OBJECTIVE
  To investigate the impact of family history on pathological and clinical outcomes after surgery for clear-cell renal cell carcinoma (ccRCC) in patients with non-syndromic disease.
  
  
  METHODS
  We reviewed 2677 patients treated with radical nephrectomy or nephron-sparing surgery for non-cystic ccRCC between 1970 and 2004 to identify patients with a family history of ccRCC. Patients with von Hippel-Lindau, tuberous sclerosis, or Birt-Hogg-Dube syndrome were excluded from analysis. Demographics and clinico-pathological outcomes were compared to patients with ccRCC without a family history of kidney cancer using chi-squared and Fisher's exact tests. Postoperative cancer-specific survival was estimated using the Kaplan-Meier method.
  
  
  RESULTS
  We identified 42 patients (1.6%) with a family history of ccRCC who were treated for non-cystic ccRCC, with a median follow-up of 4.7 years (range 1-34). Demographics and tumour characteristics, including tumour stage and grade, were similar between the two groups. Patients with a family history of ccRCC were more likely to have bilateral tumours (11.9 vs 2.2%, P= 0.003). Nevertheless, cancer-specific survival rates for patients with and without a family history of ccRCC were similar at 5 years (75.7 vs 71.1%) and 10 years (53.9 vs 62.2%).
  
  
  CONCLUSIONS
  Patients with a family history of ccRCC have pathological and clinical outcomes similar to patients with sporadic ccRCC. The increased incidence of bilateral tumours associated with a family history of ccRCC provides further evidence to support a nephron-sparing surgical approach when feasible.
authors.affiliation: Departments of Urology and Health Sciences Research, Mayo Medical School and Mayo Clinic, Rochester, MN, USA.
authors.forename: Matthew K
authors.initials: MK
authors.lastname: Tollefson
authors.forename: Stephen A
authors.initials: SA
authors.lastname: Boorjian
authors.forename: Christine M
authors.initials: CM
authors.lastname: Lohse
authors.forename: Michael L
authors.initials: ML
authors.lastname: Blute
authors.forename: Bradley C
authors.initials: BC
authors.lastname: Leibovich
mesh_terms: D000368:Aged; D002292:Carcinoma, Renal Cell / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D004812:Epidemiologic Methods; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007680:Kidney Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009392:Nephrectomy; D009399:Nephrons; D010375:Pedigree; D016896:Treatment Outcome