Renal transplantation in patients with renal cell carcinoma and von Hippel-Lindau disease

This study reviewed the management and outcome of patients with von Hippel-Lindau disease (VHLD) who underwent renal transplantation after being rendered anephric to treat multifocal bilateral renal cell carcinoma (RCC). Five patients with bilateral RCC and VHLD underwent renal transplantation at our hospital. Initial treatment of RCC consisted of bilateral nephrectomy in 2 patients and unilateral nephron-sparing surgery with contralateral nephrectomy in 3 patients. All of the latter 3 patients experienced isolated tumor recurrence in the renal remnant at 48, 64, and 66 months postoperatively; this was managed by complete excision of the renal remnant. Renal transplantation was performed 11 to 24 months after initiation of dialysis. Postoperatively, all of the allografts functioned well with no further requirement for dialysis. Currently, 4 patients are alive at a mean post-transplant follow-up interval of 26 months (range, 7 to 66 months) with excellent graft function and no evidence of malignancy. One patient died 17 months following transplantation due to metastatic disease. Renal transplantation can provide satisfactory replacement therapy for patients with end-stage renal disease with VHLD and treated RCC.