Surgical treatment of renal-cell carcinoma in patient with von-Hippel disease

Objective. To describe the clinical outcome of surgical treatment of renal-cell carcinoma in patients with von Hippel-Lindau. disease. The clinical, demographic and histopathological characteristics were evaluated. Material and methods. Of 468 patients with renal-cell carcinoma treated surgically at our institution from 1982 to 2010, eight with von Hippel-Lindau disease were identified. Relevant variables included tumor stage, type of surgery, recurrence and mid-term renal function. Results. Seven out of eight patients 7 were treated with nephron-sparing surgery. The other underwent radical nephrectomy. During the follow-up of 89.3 +/- 67.5 months, two patients had ipsilateral tumor recurrence and one in the contralateral kidney. Mean survival was 72 months in seven out of eight patients that also preserved normal renal function; one patient with uncontrolled diabetes died five years after surgery from complications of chronic kidney disease. Conclusion. Nephron-sparing surgery offers acceptable functional and oncologic results for patients with renal-cell carcinoma and von Hippel-Lindau disease.