ArticleUrologic oncology2023
Outcomes of and indications for renal transplantation in patients with von Hippel Lindau disease.
Article in Urologic oncology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 6 citations in OpenAlex.
- Completion Nephrectomy Outcomes Following Prior Partial Nephrectomy in Hereditary Kidney Cancer Syndromes.Current oncology (Toronto, Ont.) · 2026Article
- Evaluation of algorithms for predicting new baseline renal function after complex partial nephrectomy for multifocal renal tumors.Urologic oncology · 2026Article
- Impact of Hereditary Cancer Susceptibility in Solid-Organ Transplant Recipients.JCO precision oncology · 2026Article
- Synchronous giant bilateral renal tumors as initial presentation of Von Hippel-Lindau disease: Sequential surgical management and transition to renal replacement therapy.Urology case reports · 2025Article
- A New Era of Management of Von Hippel-Lindau Disease-Associated Tumors With Belzutifan.Clinical journal of oncology nursing · 2025Article
- A highly biocompatible polyethyleneimine/sulfonated polysulfone hemoperfusion microsphere with tailored surface charge for rapid and efficient removal of major protein-bound uremic toxins from simulated human plasma.Regenerative biomaterials · 2025Article
- Cumulative Impact of Serial Partial Nephrectomy for the Treatment of Recurrent Renal Masses.The Journal of urology · 2024Article
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Authors and funding
8 authors at 1 institution in 1 country.
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Abstract
INTRODUCTION AND
objectiveVon Hippel-Lindau (VHL) is a hereditary cancer syndrome characterized by bilateral, multifocal renal masses. The cumulative impact of extirpative surgery can depreciate renal function and render patients anephric. In the larger end-stage renal disease population, renal transplant offers both excellent quality of life and functional renal replacement. This case control study aims to examine and compare oncologic and functional outcomes of patients who have undergone renal transplant as renal replacement therapy (RRT) to those who remain anephric.
methodsPatient charts were retrospectively reviewed of patients with germline testing confirmed VHL between 1980 and 2022 for transplant, all prior surgical history (within and outside the NCI), renal function and graft outcomes. Overall survival (OS) was determined from years after radical nephrectomy, and graft time was defined as years of graft function from initial transplant until failure or patient death. Graft survival was determined as time between transplant(s) to last follow up. Kaplan-Meier analysis was conducted to compare graft times of anephric VHL patients to those with transplanted kidneys.
resultsA total of 23 VHLD patients were identified as either anephric or candidates for transplant. Out of this cohort, 11 total VHLD received 12 total kidney grafts. Median wait time from nephrectomy to transplant was 22.6 months (IQR: 1.02-40.25 months). Median age at transplant was 32 years (IQR: 23-54 years). OS at 5 and 10 years of anephric patients who did not receive a transplant was 33% and 16.7%, respectively. OS rates of the transplant cohort at 10, 15, and 20 years were 91%, 78%, and 58% years, respectively. Median graft time was 161 months (IQR: 56-214 months). Graft survival at 10, 15, and 20 years was 69.8%, 69.8%, and 26.2%, respectively.
conclusionsWe demonstrate that transplant recipients have decreased mortality with no difference in cancer recurrence compared to those who do not receive renal transplant for RRT. This data can aid in informing providers of the optimal window for early RRT planning in VHL, while also improving patient counseling.
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