When people hear that my sister and mother and I have all had Stereotactic Radiosurgery, or Gamma Knife surgery, they want to know – what do you think of it? Would you recommend it? Well, it depends.

We have learned a lot in the last two years about when stereotactic radiosurgery is wonderful, and when it is not a good choice. It sounds so seductive – you go in for day treatment, like having dental work, and then go have lunch in a restaurant – wow! Comparing that to going through an open surgical procedure, it sounds great. But there’s a lot they don’t tell you up front.

It was Dr. Oldfield from the National Institutes of Health who suggested to my sister Carolyn that she consider stereotactic radiosurgery (SR) for her three brain tumors. He felt they were of a size that is usually a good target size, and in a good position so that the post-operative swelling would not be a problem, and none of them had a cyst. She checked with the clinic in Memphis, and had the procedure in the fall of 1996. She had some headaches, but little negative reaction to the treatment. At her 90-day checkup, they were already seeing some improvement, some shrinkage of the tumors! We were thrilled for her – and envious.

"Do me, too," our mother said. Seeing Carolyn’s success, our mother, Pat, asked the treatment center if they could treat her two brain tumors with SR too. They did the treatment in December 1996, and the treatment went well. One of the tumors had a cyst, but our SR team felt that once the tumor shrank the cyst would shrink also. Mother was thrilled to avoid having another open brain surgery.

At Mother’s checkup in May 1997, the report from the MRI was that the tumor was gone, though the cyst was still there. Mother’s results seemed to be good, and Carolyn was still doing fine.

"Do me, too," I said. I had one hemangioblastoma with a cyst which was not causing symptoms, but this seemed such an easy way to get rid of it, I thought I might as well get it done too.

Being in the research protocol at NIH, I was in touch with some of the nurses and researchers there, and the word got back to Dr. Oldfield. The day before the procedure, Dr. Oldfield called me, and said he thought we should talk more about stereotactic radiosurgery before I went ahead with the treatment. While Carolyn’s tumors were just the right size and placement for successful treatment, mine, he said, with its cyst, was not a good candidate for stereotactic radiosurgery. While the treatment might shrink the tumor, it would not do so immediately, and there was danger that the cyst might grow more before the tumor was controlled. He warned me that there was danger for me in proceeding with this treatment, that for this particular kind of tumor he would recommend open surgery.

Like so many other people with serious illness, I was in a quandary. I had one physician whom I greatly respect telling me there was danger. I had another physician, at the gamma knife center, telling me he could successfully treat the lesion. My mother and sister had had good outcomes, and my mother’s lesion had a cyst. How was I to make the decision? I read articles in Medline. There was one sample of ten patients with cystic hemangioblastoma. Six had problems following SR, two had urgent problems, one needed emergency surgery. But, I rationalized, ten is not a very big sample, and the bad outcomes wouldn’t happen to me.

I called the physician who would perform my SR treatment to tell him about Dr. Oldfield’s call. He conducted research of how own, consulting the national SR database. He read the article I had found in Medline. He found no other data indicating that cystic hemangioblastomas were not good candidates for SR. He urged me to continue with my plans.

I felt committed to go forward the following day with the scheduled procedure. And most of all, I wanted it to be successful. I wanted to believe that I could have the same success my mother and sister were enjoying. I didn’t want to have surgery, didn’t want a "zipper" in the back of my head. So I went ahead with the procedure, went out to lunch after the procedure, and felt I had made the right decision.

Then the nightmare began.

In June 1997 my mother began to have headaches – big headaches. They prescribed steroids, and kept increasing the dosages, but it wouldn’t stop. There was more and more pressure in her head. Her brain was swelling. Over the summer on several occasions she had serious reactions to the large amounts of decadron she was taking. They changed the medication, but her brain continued to swell. Headaches, double vision, difficulty walking – things we now know were caused by the long-term swelling reaction from the radiosurgery. The steroids caused problems of their own – heavy dosages, reactions to medication, rapid weaning off the medication and reactions from that withdrawal, diverticulitis – altogether a bad scene. Mother was now completely bedridden and in the hospital.

With all the crises around my mother’s health, I didn’t go for my three-month checkup. After all, I was feeling fine, and it was Mother we were worrying about. By September I too started to have headaches – bad ones – and on October 23 they did surgery to remove a 31 mm cyst from my brain. The cyst had continued to grow and had to be surgically removed after all.

Mother was having incredible pain. At first it was blamed on a "bad pillow" or other normal hospital complaints, but finally my sister and I convinced the hospital that Mother was not a complainer, and they should do an MRI. Once they took a look at the MRI they scheduled emergency surgery to remove the tumor and the enlarged cyst, which had continued to grow. She continues to have dizziness and weakness in her legs, and her brain still shows signs of swelling, 17 months after the SR procedure. She is still being weaned from the steroids.

What we learned was that stereotactic radiosurgery can be wonderful, or can be terrifying. Thanks to learning accumulated over the past five years of experience at a number of SR treatment centers, by NIH, and by the VHL Family Alliance members sharing their experiences, there are now some pretty good guidelines for when SR will likely be successful, and when it is inadvisable.

The guidelines agreed at the Bethesda Focus Meeting on Stereotactic Radiosurgery are that the hemangioblastomas that respond best to SR are 10-12 mm. or smaller and where there is no cyst creating pressure inside the skull. [see also Adler]

It is important to understand how it works. You have to know too that hemangioblastomas are very rare tumors, and the amount of experience most centers have in treating hemangioblastomas is very limited. Hemangioblastomas account for only two percent (2%) of all brain tumors. And hemangioblastomas react very differently to SR than hard tumors. That is why it is critically important that we share our experiences with the Alliance, and make sure that all these outcomes – both good and bad – contribute to our learning and create better outcomes for people in the future. For example, adverse reactions to the drugs, like decadron, had not been reported in papers about stereotactic radiosurgery, because the procedure had gone well: it was the drug that caused the problem. The Alliance, however, has collected information about four such cases. Remembering that there are not yet 100 patients who have had stereotactic radiosurgery for hemangioblastoma, four is a significant number. Through the Alliance, we can share the real human experience of the procedure, and whether we as humans feel the procedure was a success. How much of an interruption was this procedure in our lives? Did we get the outcome we wanted? Was it worth it?

Essentially, SR zaps the tumor with beams of radiation from hundreds of different angles, so that as each beam passes through normal tissue that tissue gets only a very small dose of radiation. Where the beams meet at the target site, the tumor gets the sum of the dosages of all the beams, the "surgical dose", which is essentially a radiation burn that intends to kill the tumor. But it doesn’t happen immediately. As with any burn, it swells for a while, then heals, and hopefully leaves the tumor dried up and dead. With burns you’ve had in the kitchen, the swelling goes on for a few hours, or a few days, and then begins to heal. But with a radiation burn, the cycle of swelling and healing can go on for months, or even years, depending on the dosage delivered. Hard tumors simply dry up and crumble; but hemangioblastomas swell.

There are ways to reduce the amount of swelling. Sometimes they will "fractionate" the dosage, or divide it across a number of sessions. Dr. Haring J. W. Nauta compares the effect to the difference between spending two hours on the beach in Miami on the first day of your winter vacation, versus spending 10 minutes in the sun twice a day for the week. You will have spent that same two hours at the beach, and had the same exposure to the sun’s radiation, but you will probably not burn. This is "fractionating" your dosage of the sun’s radiation.

I encourage you to talk with your doctors – a serious, open-minded talk – with both a neurosurgeon and a radiation oncologist, and ask them all the hard questions that are in the VHL Handbook. Those questions are there for a reason – they represent the hard learning of families like mine whose outcomes were not so great because they didn’t have all the information up front. Don’t be pig-headed, as I was. I wanted to avoid surgery – I had it anyway. I got my zipper after all, but it’s well hidden by my hair, and it’s not so bad.

Get a second opinion, or even three. Ask one of the doctors with experience in treating hemangioblastoma to review the treatment plan before, not after it happens. Once the radiation dose has been delivered, they can’t take it back. You’re on the roller-coaster, and you have to do the whole ride.

And if you do have the procedure, be very sure to go back for follow-up as scheduled, don’t minimize the importance of those follow-ups, even if you’re feeling good. Make sure they are monitoring the pressure inside the skull, especially if you experience headaches, vision disturbance, or other neurological symptoms. With careful monitoring, there is a better chance of controlling negative consequences.

Carolyn continues to do well. For her, sterotactic radiosurgery was indeed the right choice. Her checkups show that the tumors are drying up as planned.

I’m back to work and doing fine after my brain surgery, six months after SR. In retrospect, my cystic lesion should not have been treated with SR. While the radiation may not have caused the cyst to grow, it also did nothing to slow down its natural tendency to grow. The best alternative for me from the beginning was open surgery.

My mother continues fragile and in poor health, though she is coming back slowly from a nearly fatal experience, and four months in the hospital, beginning six months after SR. Of course there is no way to know what might have happened if she had had open brain surgery in the first place, but it could not have been any worse than this.

[new Adler article published July 1998]

We think that patients with VHL disease who have had their diagnosis established and who present with small (<3 cm) solid hemanbioblastomas without significant mass effects are reasonable candidates for radiosurgery... Microsurgical resection remains the treatment of choice for the vast majority of symptomatic cystic hemangioblastomas .. because of the need to eliminate mass effects ... For rare patients with very numerous lesions, treatment is limited to symptomatic and radiographically enlarging tumors, to minimize excessive radiation. -- Chang, Adler, et al., "Treatment of Hemangioblastomas in von Hippel-Lindau Disease with Linear Accelerator-based Radiosurgery," Neurosurgery 43:1, July 1998.

See Questions to Ask the Doctor about Stereotactic Radiosurgery

As printed in the VHL Family Forum 6:2, June 1998. For permission to reprint, please contact VHL Family Alliance, info@vhl.org.