RAPN can provide tumor control while preserving renal tissue in patients with VHL-associated multifocal RCC and a solitary kidney, but the procedure carries a substantial risk of severe postoperative AKI requiring temporary renal replacement therapy.
Key Findings
Results
A transperitoneal robotic-assisted partial nephrectomy achieved complete excision of all tumors with negative surgical margins in a 37-year-old man with VHL disease and a solitary right kidney.
The patient carried a VHL c.257C>G, p.P86R mutation and had previously undergone left radical nephrectomy.
He presented with three progressive clear cell RCC lesions, the largest measuring 4.8 cm, despite ongoing belzutifan (HIF-2α inhibitor) treatment.
A fourth tumor was identified intraoperatively and was also excised.
Surgical margins were negative for all tumors.
Results
The patient developed severe postoperative acute kidney injury requiring continuous renal replacement therapy.
Renal function gradually recovered following intensive care management.
CRRT was discontinued on postoperative day 8.
The authors conclude the procedure carries a substantial risk of severe postoperative AKI requiring temporary renal replacement therapy.
Results
CRRT was complicated by forearm necrosis resulting from paravasation of calcium substitution.
The necrosis was attributed to calcium substitution paravasation during CRRT.
This was reported as an additional complication of the AKI management.
Results
The patient also developed a right pneumothorax postoperatively, managed with pleural drainage.
Management took place in intensive care, including pleural drainage.
Renal function recovered after intensive care management.
Results
Tumors progressed in this VHL patient despite ongoing belzutifan therapy.
Three ccRCC lesions were progressive while the patient was on belzutifan, a HIF-2α inhibitor.
The largest lesion measured 4.8 cm.
Background
Nephron-sparing surgery in VHL patients with a solitary kidney is aimed at preserving renal function to prevent end-stage renal disease and dialysis dependence.
VHL disease is a rare autosomal dominant tumor syndrome caused by germline VHL mutations.
Patients may develop multiple, bilateral, and recurrent RCCs, which poses a major therapeutic challenge, especially with a solitary kidney.
What This Means
This report describes a 37-year-old man with von Hippel-Lindau disease, an inherited condition that causes multiple kidney cancers. He had already lost his left kidney to surgery, so his remaining right kidney was critical. Despite taking belzutifan, a targeted drug, three kidney tumors kept growing, the largest 4.8 cm. Surgeons used a robotic-assisted approach to remove the tumors while keeping as much of the kidney as possible. A fourth tumor was found during surgery and was also removed, and all tumors were removed completely with clean margins.
Recovery was difficult. The patient developed severe acute kidney injury and needed temporary kidney replacement therapy (continuous dialysis-type treatment). A calcium solution leaked outside a vein during this treatment and caused tissue death in his forearm. He also developed a collapsed lung (pneumothorax) that needed a drain. With intensive care his kidney function gradually recovered, and the dialysis-type treatment stopped on day 8 after surgery.
This research suggests that robotic partial removal of kidney tumors can control cancer while sparing kidney tissue in people with VHL and a single kidney, potentially helping them avoid permanent dialysis. However, it also shows the operation can cause serious short-term kidney injury and other complications. This is a single case report, so it cannot show how often these outcomes occur.
Farzat M, Al-Taie I, Wagenlehner F. (2026). Robotic-Assisted Partial Nephrectomy in a Solitary Kidney in a von Hippel-Lindau Renal Cell Carcinoma.. Journal of visualized experiments : JoVE. https://doi.org/10.3791/71597