J Vis Exp. 2026 Oct 30;(236). doi: 10.3791/71597.
ABSTRACT
Von Hippel-Lindau (VHL) disease is a rare autosomal dominant tumor syndrome caused by germline mutations in the VHL gene. Patients may develop multiple, bilateral, and recurrent renal cell carcinomas (RCCs), posing a major therapeutic challenge, particularly in those with a solitary kidney, where preservation of renal function is critical to prevent end-stage renal disease and dialysis dependence. This case demonstrates the surgical management of multiple renal tumors in a solitary kidney. A 37-year-old man with VHL disease (c.257C > G, p.P86R mutation) and a solitary right kidney following previous left radical nephrectomy presented with three progressive clear cell RCC (ccRCC) lesions, the largest measuring 4.8 cm, despite ongoing treatment with belzutifan, a HIF-2α inhibitor. A transperitoneal robotic-assisted partial nephrectomy (RAPN) was performed. A fourth tumor was identified intraoperatively. Complete excision of all tumors with negative surgical margins was achieved. Postoperatively, severe acute kidney injury (AKI) developed, requiring continuous renal replacement therapy (CRRT), which was complicated by forearm necrosis resulting from calcium substitution paravasation. An additional complication included a right pneumothorax. Following intensive care management, including pleural drainage, renal function gradually recovered, and CRRT was discontinued on postoperative day 8. This case demonstrates that RAPN can provide tumor control while preserving renal tissue in patients with VHL-associated multifocal RCC and a solitary kidney. However, the procedure carries a substantial risk of severe postoperative AKI requiring temporary renal replacement therapy.
PMID:42965379 | DOI:10.3791/71597