Renal cell carcinoma is among major causes of death in patients with Von Hippel-Lindau (VHL) syndrome, and it usually presents with multiple and bilateral lesions that may require multiple renal surgeries. This, in turn, may compromise renal function, resulting in end-stage renal disease. To minimize renal function impairment in these patients, great importance is given to the preservation of functional parenchyma with the use of nephron-sparing techniques. Furthermore, new techniques such as off-clamp surgery, selective suturing or sutureless techniques may improve long-term functional outcomes. We described the case of a 27-year-old male patient with a family history of VHL disease affected by multiple, bilateral renal masses. He received bilateral, metachronous robot-assisted partial nephrectomies (RAPN) for a total of 15 renal lesions. No intra- or post-operative complications occurred, and the patient was discharged on the second postoperative day after both procedures. Serum creatinine after the second RAPN was 0.99 mg/dl (baseline value was 1.11 mg/dl). In patients with VHL syndrome and multiple renal lesions, robot-assisted partial nephrectomy, especially with the use of clampless and sutureless techniques, helps minimizing renal function impairment and should be performed when anatomically and technically feasible.
Keywords: Familial renal cancer; Nephron-sparing surgery; Partial Nephrectomy; Robotic surgery; Von Hippel–Lindau disease.
© 2023. The Author(s) under exclusive licence to The Japan Society of Nephrology.