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Patterns of intervention for renal lesions in von Hippel-Lindau disease
Surena F Matin1, Kamran Ahrar, Christopher G Wood
1Department of Urology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. surmatin@mdanderson.org
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.
Insights
Von Hippel-Lindau (VHL) disease patients with kidney tumors often require intervention. Open partial nephrectomy (OPN) is the primary treatment, with radiofrequency ablation (RFA) used less frequently due to technological limitations.
Area of Science:
- Nephrology
- Oncology
- Genetics
Background:
- Von Hippel-Lindau (VHL) disease is a genetic disorder predisposing individuals to various tumors, including renal cell carcinoma (RCC).
- Management of VHL-associated RCC often involves complex surgical decisions to preserve renal function.
Purpose of the Study:
- To review VHL disease patient records to determine RCC incidence.
- To analyze intervention patterns for renal tumors in VHL patients, focusing on minimally invasive techniques.
Main Methods:
- Retrospective review of genetically confirmed VHL patients treated at a multidisciplinary center.
- Evaluation of interventions including percutaneous radiofrequency ablation (RFA) and open partial nephrectomy (OPN).
- Contraindications for RFA included cystic tumors, large tumors (>3 cm), or extensive multifocality.
Main Results:
- Of 38 VHL patients, 16 (42%) had RCC; 14 underwent 25 renal interventions.
- Open partial nephrectomy (OPN) was performed in 60% of cases, RFA in 20%.
- Median follow-up of 41 months showed 93.3% metastasis-free survival and 87.5% overall survival, with no progression to end-stage renal disease.
Conclusions:
- 88% of VHL patients with renal involvement require intervention.
- OPN is the primary successful intervention for VHL-associated renal tumors.
- Targeted therapies may reduce the need for surgical interventions in VHL patients.
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