Eva’s death was caused by metastatic cancer throughout her body. One kidney tumor grew to 5 centimeters, moved outside the kidney capsule, and metastasized.

Nearly every day for the past month people have called the hotline to ask, "How could this happen? And to Eva, of all people? Eva is the one who has been coaching me in managing my health!"

We have seen it before — Adele Davis was one of the first proponents of nutrition for cancer prevention. When her husband died of cancer in his 70’s many people took that as an opportunity to question her entire philosophy. Dr. Jim Fixx, the first big advocate of running and aerobic exercise to take care of your heart, died of a heart attack. Such things call the strategy into question, and it is always good to examine and learn from such sad events. But just as their strategies proved to be good ones, the strategies that Eva taught — those which are to be found in the VHL Handbook — are still what we need to follow. And we defiinitely need to learn more!

In the spirit of learning, we posed some key questions to our medical advisors:

Question: We have a "guideline" that says we can wait until the tumor reaches 3 cm before taking action. We know that’s not foolproof, but is a rough measure of tumor activity. The kidney tumor in question seemed to grow more quickly than expected — growing in six months from 2.5 cm to 5 cm. We know that size is only a crude estimate of the danger level of a kidney tumor — what we really want to measure is its activity level. Are there any better ways to measure the activity level of a tumor?

Answer: Not yet, but this is an active area of research for all cancers. It’s the same question we have in the pancreas, the breast — everywhere that cancers can grow, there can also be benign growths, or early growths, that require more or less action depending on their levels of activity. When activity levels are low, less invasive actions can be taken that will solve the problem with little impact on the patient. When activity levels are high, more aggressive action should be taken even if there is more surgical impact, because the impact of not acting would be more destructive than the impact of the surgery.

There are modalities currently in clinical trials that should help us — for example, PET markers to show on a scan the level of VEGF production surrounding the tumor. Once we have this kind of tool available, we will be able to monitor a tumor more closely and more often with less cost, less radiation exposure, and more helpful information.

Question: When the 5 cm tumor was removed, they had to remove some back muscle to get a clean margin. Clearly at this point it was known that the tumor was outside the kidney capsule, and the pathology report showed that it was a grade 3-4 tumor (with high metastatic potential). In a case like this should there be follow-up radiation or chemotherapy? How frequently should you have check-ups to check for metastasis?

Answer: The situation you describe is already dangerous. These are good questions that can only be answered in conference with your own medical team, with all the scans, pathology information, and the total medical picture at hand. If you are not comfortable or confident with the answers being given locally, do not hesitate to seek a second opinion, especially from a team with greater experience in VHL. Chemotherapy itself can be a difficult course, has a low chance of success, and is not for everyone. Further surgery may or may not be helpful.

Question: Eva had had a difficult recovery from a prior kidney surgery, and dreaded having open surgery again. She wanted to have Radio Frequency Ablation, not open surgery. This relatively large tumor did not respond to RFA treatment, but continued to grow. What should we know about qualifying a kidney tumor for RFA treatment versus open surgery?

Answer: It is still early in our learning about radiofrequency thermal ablation (RFA). This minimally invasive, image-guided therapy may now provide effective local treatment of VHL tumors, and can also be used in other cancer tumors as an adjunct to conventional surgery, systemic chemotherapy, or radiation. We have the greatest amount of data for liver cancer, and have treated 24 kidney tumors including 19 patients with VHL (see Note 1). While nearly all patients came through the treatment itself very well, we do not yet know how well the tumor will be inactivated over the long term. This treatment will remain experimental until we have a better grasp on ways to measure tumor destruction.

If a target can be seen with CT, MR, or ultrasound, then a needle can be placed into it. If a needle can be placed, then the target tissue or tumor can be ablated and destroyed. If a clean margin is created, then the tumor should not recur at that site. Recent developments in RFA allow this treatment process to be done in a safe, predictable, and cheap fashion with low complication rates and minimal discomfort, on an outpatient basis. Further study is required to assess which patients will benefit from this new treatment, and most patients will not be candidates due to the size or location of the tumor. Although long-term data have yet to be reported, early results suggest that RFA may prove to be an effective treatment option for small kidney tumors (under 3 cm), disabling them before they grow to larger sizes. The standard of care for tumors larger than 3 cm continues to be surgical removal of the tumor.

As Eva’s husband Chris said, "In 20/20 hindsight, I feel our medical system has become so specialized in their fields of expertise that the patient has to now act as their own general health coordinator. In too many cases the general practitioner just shuffles you off to the various specialists and doesn’t fulfill the role of coordinator needed to protect the patient." Get copies and read your radiology reports and pathology reports. Take someone with you to appointments. Don’t depend on understanding everything the doctor tells you after surgery while you are still in pain and on drugs. Read the reports again a few weeks later, and ask all your questions. Don’t be shy. The doctor has many patients, but you have only one body. It’s your responsibility, even more so that the doctor, to take charge of your own health.

"Even though Eva wasn’t given the opportunity to grow old as gracefully as I would have liked," says Chris, "she did receive better healthcare than the generation before her. Her ability to get more and more information concerning VHL did permit her to minimize or alleviate the affects this disease has the potential to inflict on the individual. Unfortunately, she didn’t beat the disease, but she certainly made her mark on improving people’s lives. She left us much to treasure."

Note 1: B. Wood et al., "Percutaneous tumor ablation", American Cancer Society (2002). C. Pavlovich et al, "Percutaneous tumor ablation of small renal tumors: Initial Results", Journal of Urology 167:10-15, January 2002.