Physicians at eight prominent U.S. medical centers pooled their findings on the treatment of 65 patients with VHL kidney tumors over the last fifteen years to determine the effectiveness of different approaches to treatment. The key question was whether it was better to deal aggressively with tumors, removing the entire kidney, or to remove only the tumors and leave the remainder of the kidney, preserving kidney function. Opinions have differed widely on this point.

VHL tumors of the kidney are closely related to the kinds of "sporadic" renal cell carcinoma (RCC) tumors which occur in about 23,000 people each year in the general population. But they are different in many significant ways. In VHL, there tend to be more tumors, usually on both kidneys, and occurring at a younger age. They tend not to grow as rapidly as in sporadic RCC. In the general population, the average age of onset of kidney cancer is 61; in VHL, it was report by Maher et al. as 44 years. In this study, the mean age of diagnosis was 36 years. With better diagnostic methods and more careful screening programs for people at risk, VHL kidney tumors are being found at younger and younger ages.

While it is important to avoid the risks of cancer spreading, or metastasizing, to other places in the body, it is also important to design a life treatment plan which will keep those with VHL healthy throughout their lives, and preferably on their own kidney power. This study looked back at the results 5-15 years after surgery, to see how patients fared with the different kinds of treatment.

They confirmed that the keys to managing VHL kidney tumors are to find the tumors early, deal with them appropriately, and keep up a conscientious program of follow-up.

They found that the 49 people who had "nephron-sparing" or kidney-saving surgeries did very well. Every one of these patients was alive five years after surgery, and 81% ten years later. While 51% of these patients had more kidney tumors within the next 5-15 years, only 2 had metastastatic cancer. This is very different from sporadic RCC, where recurrence usually means the cancer has spread. Thirteen of these people had an additional nephron-sparing surgery, 6 have not required additional treatment yet, and 6 have had a complete removal of the remaining kidney. Recurrence is likely due to microscopic RCC that was not removed in the initial operation.

Most of the patients in the study (54 out of 65) had tumors on both sides. Most of the tumors were found during routine screening; 37% were found because of symptoms, which usually means they already had more extensive involvement. Only one patient had metastatic disease when the kidney tumors were diagnosed.

A total of 68 kidneys in 49 patients were treated with nephron-sparing surgery, removing the tumors and leaving as much functioning kidney as possible. Eight patients were treated with removal of one entire kidney, and another 8 with removal of both kidneys. After surgery, all patients returned for check-ups on a regular basis for up to 11 years.

In the course of the study, 15 out of 65 patients lost kidney function and required dialysis. Six of these had kidney transplants within two years of beginning dialysis. All the grafts were successful. Nine of the patients have been managed with dialysis alone.

This study suggests that "when nephron sparing surgery is technically feasible, this approach can preserve renal function for an extended interval without compromising cancer-free survival in most patients. However, patients treated in this manner should be advised of the importance of close postoperative surveillance and probable need for repeat renal surgery in the future." Recurrence typically does not happen for several years, and the risk of having an associated metastasis appears to be small.

When patients were diagnosed due to symptoms, they usually already had more extensive involvement. This underscores the need for pre-symptomatic screening in people with VHL at risk for kidney tumors. Where the condition was diagnosed early, more conservative treatment had a much better chance of success.

Where removing all remaining kidney tissue is required, kidney transplantation can be used successfully as effective replacement therapy.

This multi-center study, headed by Dr. Andrew C. Novick, Chairman of the Department of Urology at the Cleveland Clinic Foundation, included sixty-five patients from the Cleveland Clinic (19), the Mayo Clinic (18), the University of Iowa (11), the University of Southern California (6), Brigham & Women's Hospital, Boston (4), U.C.L.A. (3), Johns Hopkins (2), and the University of Washington (2).

Frank Steinbach, Andrew C. Novick, Dave P. Miller, Horst Zincke, Richard D. Williams, Greg Lund, Donald G. Skinner, David Esrig, Jerome P. Richie, Jean B. deKernion, Fray Marshall, and Christopher L. Marsh, "Treatment of Renal Cell Carcinoma in von Hippel-Lindau Disease: A Multi-Center Study." Journal of Urology.

As printed in the VHL Family Forum 3:2, June 1995:. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.