Patterns of intervention for renal lesions in von Hippel-Lindau disease.

OBJECTIVE

To review the records of patients at our centre with von Hippel-Lindau (VHL) disease, to determine the incidence of renal cell carcinoma (RCC) and patterns of intervention using minimally invasive therapies.

PATIENTS AND METHODS

Patients with genetically confirmed VHL were evaluated in a multidisciplinary clinical care centre established in 2003. Patients were preferentially offered percutaneous radiofrequency ablation (RFA). Cystic tumours were considered contraindications to RFA, as were larger tumours or extensive multifocality with tumours of >3 cm. These patients had either open partial nephrectomy (OPN) or, in unsalvageable cases, radical nephrectomy.

RESULTS

Of 38 patients with VHL, 16 (42%) were found to have RCC; two with small tumours are under observation. Fourteen of the 16 have had a total of 25 renal interventions, none of whom has progressed to end-stage renal disease. OPN was performed in 15 (60%) cases, including those who had had multiple bilateral procedures; RFA was used in five (20%) cases. After median follow-up of 41 months, local recurrence was detected in 33%; the metastasis-free survival rate was 93.3% and overall survival 87.5%.

CONCLUSIONS

Of patients with VHL, 88% with renal involvement require interventions for their kidneys. OPN is the primary method used, and was successful both as a primary and secondary procedure in 60% of cases. In only 20% was RFA possible due to limitations of current technology. The introduction of protocol-based targeted therapies holds the promise of reducing the number of interventions required for treating VHL.