*Purpose: Von Hippel-Lindau disease is a hereditary cancer syndrome characterized by bilateral, multifocal renal tumors and cysts. Advancements in nephron-sparing surgeries have improved functional and oncologic outcomes in these patients. If parenchyma cannot be salvaged, renal replacement therapy is indicated. This case series aims to examine outcomes of patients who have undergone renal transplant secondary to surgical loss their kidneys.

*Methods: We identified 8 VHL patients in an institutionally maintained database at the NCI undergoing renal transplant from 1989 to 2021. Transplants were conducted at an outside institution for all patients, and charts were reviewed for surgical and transplant outcomes. Graft time was defined as years of graft function until failure or patient death.

*Results: Median partial nephrectomies prior to radical nephrectomy was 2 (IQR: 1.25-3.5). Three patients underwent bilateral nephrectomy with no prior partial nephrectomy. At time of transplant, seven patients had no native tissue found on imaging of graft, while one patient had partial native kidney. Three patients had data available for donor kidney source, with two having cadaveric donors, and one with a living donor. All patients exhibited signs of renal failure at time of transplant, with complications such as uremic cardiomyopathy (n=1) and electrolyte derangements (n=4). Patients spent a median of 3.5 years on dialysis, consistent with the national average of ‘time on transplant list’ at 3.6 years. Median age at transplant was 28, ranging from 23 to 67. Median graft time was 5 years, with a graft range of 1 to 20 years. All the patients were prescribed immunosuppressive therapy with prednisolone and immunomodulators, most commonly tacrolimus. All patients tolerated immunosuppression, but one patient who developed systemic fungal infection and died. One patient developed an acute graft rejection, diagnosed on allograft biopsy months after the transplant. One patient died during partial nephrectomy of native kidney. None of the subjects developed tumor recurrence or metastatic disease on imaging.

*Conclusions: Given the operative challenges posed by VHL, transplant is a viable option for patients with limited functional kidney parenchyma. Though renal transplant is minimally utilized in this institutional cohort, this case series suggests that risk of disease recurrence in graft and development of metastasis is minimal. This demonstrates utility of renal transplant in management of end-stage renal disease within the VHL patient population.

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