title: [Surgical treatment of renal-cell carcinoma in von Hippel-Lindau disease].
abstract: OBJECTIVE
  To describe the clinical outcome of surgical treatment of renal-cell carcinoma in patients with von Hippel-Lindau disease. To analyze the clinical, demographic and histopathological characteristics.
  
  
  METHODS
  From a total of 468 patients with renal-cell carcinoma treated surgically at our institution from 1982 to 2010, a series of 8 with von Hippel-Lindau disease were identified. Relevant variables included tumor recurrence, surgical approach, recurrence rate and mid-term preserved renal function.
  
  
  RESULTS
  Eight patients with von Hippel-Lindau disease and renal cell carcinoma were identified; 7 were treated with nephron-sparing surgery and 1 with radical nephrectomy. During the follow-up of 89.3 ± 67.5 months, we found ipsilateral tumor recurrence in two patients and contralateral in another one. Mean survival time was 72 months in seven out of eight patients who also preserved normal renal function; one patient with uncontrolled diabetes died from complications of chronic kidney disease five years after surgery.
  
  
  CONCLUSIONS
  Nephron-sparing surgery offers acceptable oncologic and functional results for patients with renal cell carcinoma and Von Hippel-Lindau disease.
authors.affiliation: Departamento de Urología, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán.
authors.forename: Jaime
authors.initials: J
authors.lastname: Uscanga-Yépez
authors.forename: Francisco
authors.initials: F
authors.lastname: Rodríguez-Covarrubias
authors.forename: Juan
authors.initials: J
authors.lastname: Morales
authors.forename: Julio
authors.initials: J
authors.lastname: Granados
authors.forename: Fernando
authors.initials: F
authors.lastname: Gabilondo-Navarro
mesh_terms: D000328:Adult; D002292:Carcinoma, Renal Cell / Q000150:complications* / Q000601:surgery*; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications* / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012189:Retrospective Studies; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000235:genetics