Abstract
From 1981 to 1986, 10 patients with von Hippel-Lindau’s disease underwent an operation for bilateral nonmetastatic renal cell carcinoma. Of the patients 9 underwent unilateral partial nephrectomy and contralateral radical nephrectomy, and 1 underwent bilateral nephrectomy with subsequent hemodialysis and cadaver renal transplantation. Pathologically, the tumor was stage I in 9 patients and stage III in 1. Currently, 8 patients are alive after partial nephrectomy (5 to 56 months) with good renal function (mean serum creatinine 2.0mg. per dl.) and no evidence of malignancy; 1 of these patients underwent excision of a cerebral metastasis 2 years after partial nephrectomy. One patient is alive on dialysis after removal of the renal remnant for local tumor recurrence. The patient who underwent transplantation is free of tumor with a well functioning allograft. The distinctive features of renal cell carcinoma in von Hippel-Lindau’s disease that influence the management of these patients are reviewed.
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