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Parenchymal sparing surgery in patients with hereditary renal cell carcinoma
M M Walther1, P L Choyke, G Weiss
1Urologic Oncology Section, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.
Abstract:
The von Hippel-Lindau syndrome is the most well known cause of familial renal cancer. Because affected individuals with renal lesions can have complex, multisystem manifestations of von Hippel-Lindau disease, our renal management strategy has included parenchymal sparing surgery whenever possible. From May 1988 to January 1993, 20 patients with hereditary renal cell carcinoma (19 with von Hippel-Lindau disease and 1 with hereditary papillary renal cancer) underwent renal exploration with the intent of performing parenchymal sparing surgery. A total of 7 nephrectomies and 27 parenchymal sparing procedures was performed. Additional procedures performed included 2 bilateral adrenalectomies for pheochromocytomas, 1 resection of a renal vein thrombus and 1 resection of a pancreatic islet cell tumor. Renal atrophy occurred in 3 of 27 kidneys (11%) treated by parenchymal sparing surgery. In 8 kidneys of 7 patients new solid lesions developed and in 14 kidneys of 12 patients no new solid lesions developed during a mean followup of 26 months (range 6 to 60 months). The use of parenchymal sparing surgery in patients with familial forms of kidney cancer is based on a desire to maintain renal function as long as possible while reducing the risk of metastases. The potential advantages and disadvantages of more ablative surgical treatment requiring subsequent dialysis or transplantation in patients with existing or potential central nervous system, eye, pancreas and/or adrenal tumors must be weighed against the possibility of renal cancer metastases or recurrence when deciding on the use of parenchymal sparing surgery.
Insights
Parenchymal sparing surgery is a viable option for managing familial kidney cancer, aiming to preserve renal function. This approach requires careful consideration of potential risks like cancer recurrence versus the benefits of maintaining kidney health.
Area of Science:
- Oncology
- Nephrology
- Genetics
Background:
- Von Hippel-Lindau (VHL) syndrome is a primary genetic cause of familial renal cancer.
- Patients with VHL disease often present with complex, multisystemic conditions.
- Management of renal lesions in these patients necessitates strategies that preserve kidney function.
Purpose of the Study:
- To evaluate the efficacy and outcomes of parenchymal sparing surgery for hereditary renal cell carcinoma.
- To assess the oncologic and functional results of kidney-sparing procedures in VHL patients.
- To compare parenchymal sparing surgery with more radical approaches in familial kidney cancer.
Main Methods:
- A retrospective review of 20 patients with hereditary renal cell carcinoma (19 VHL, 1 hereditary papillary renal cancer) from May 1988 to January 1993.
- Surgical interventions included 7 nephrectomies and 27 parenchymal sparing procedures.
- Follow-up included monitoring for new solid lesions, renal atrophy, and oncologic outcomes over a mean of 26 months.
Main Results:
- Renal atrophy occurred in 11% (3/27) of kidneys treated with parenchymal sparing surgery.
- New solid lesions developed in 8 kidneys (from 7 patients) during follow-up.
- No new solid lesions were observed in 14 kidneys (from 12 patients) over the follow-up period.
Conclusions:
- Parenchymal sparing surgery is a valuable approach for preserving renal function in patients with familial kidney cancer, particularly those with VHL syndrome.
- The decision to use parenchymal sparing surgery involves balancing the risk of cancer recurrence against the benefits of maintaining kidney function and avoiding dialysis or transplantation.
- Careful patient selection and surgical planning are crucial for optimizing outcomes in hereditary renal cell carcinoma management.