Conservative renal surgery for renal cell carcinoma in von Hippel-Lindau's disease.

A total of 19 patients with renal cell carcinoma associated with von Hippel-Lindau's disease is described. Of the 7 patients with unilateral disease of low grade and stage 6 were treated with a renal sparing operation and 1 with radical nephrectomy. They had no evidence of disease during followup of up to 12 years (mean 4.8 years). Of the 12 patients with bilateral disease 3 presented with tumors of low grade and stage, and were treated successfully with a conservative operation. In the remaining 9 patients disease of high grade and stage had progressed and 6 died. Conservative renal operation is best suited for the typical tumor of low grade and stage, and preferably unilateral disease, associated with von Hippel-Lindau's disease. For bilateral disease of low grade and stage a similar conservative approach may be considered with caution. However, in the presence of high stage and multicentric tumors, most patients with bilateral tumors are probably better served by radical nephrectomy and subsequent renal replacement therapy.