[Surgical treatment of renal-cell carcinoma in von Hippel-Lindau disease]. / Tratamiento quirurgico del carcinoma de celulas renales en la enfermedad de von Hippel-Lindau.

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. MATERIAL AND 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.