It is still perhaps the most dreaded diagnosis but methods of treating it are improving.
Jordan Fieldman was a 23-year-old first year student at Harvard Medical School when he was told that a brain tumor would probably kill him before the year was out.
For five years, he’d had “horrendous headaches” that were written off as stress, he says. He’d also had trouble seeing what was on the blackboard since his undergraduate days as a neuroscience major at Harvard.But the student health plan, he says, kept referring him to an optometrist, who told him to keep using his glasses.
So it wasn’t until medical school that he saw an ophthalmologist, an eye doctor, who detected a brain tumor and rushed Fieldman to the hospital in an ambulance. He underwent a 15-hour operation to remove the tumor from the pineal gland in the middle of his brain. The surgery left him temporarily blind.
Still, Fieldman kept going to class – “If I went home, I’d be a cancer patient; if I stayed in school, I’d be a medical student who happens to disappear for treatment” – while going through nine months of experimental chemotherapy and radiation.
He was given “nonexistent” odds of living five years, but he beat those odds. He is 31 now, a doctor at the Berkshire Medical Center in Pittsfield.
Despite such encouraging stories, there is perhaps no diagnosis more frightening than a brain tumor, and according to The Brain Tumor Society, a national group based in Boston, it is a diagnosis that roughly 100,000 Americans will hear every year.
Worse still, say some experts and activists, the incidence of such tumors may be on the rise, though this is a hotly disputed point. There is consensus, though, that in recent years, there have been significant advances in treatment.
The fear that incidence may be rising has galvanized activists. Last week, more than 150 of them met with members of Congress to lobby for more funding. And this Saturday, hundreds of patients, family members and health professionals are expected to gather at a Boston symposium to discuss the latest research on treatment.
Most tumors in the brain are cancers that spread there from other organs in the body. But every year, about 34,000 people are diagnosed with “primary” tumors that arise in the brain itself, according to the Central Brain Tumor Registry of the United States, the only organization that keeps track of both malignant and benign brain tumors.
Even benign tumors can be life threatening, depending on their location. Slightly more than half of all primary tumors are malignant.
According to the National Cancer Institute, which keeps track only of malignant tumors, the incidence of primary brain tumors rose 18.5 percent between 1973 and 1994. The biggest increases seem to be at the extremes of life – a 57.9 percent increase in people over 65, and a 35 percent jump in children from birth to 14.
Other researchers, however, question whether the rate is really going up, or it’s just that detection is better.
To be sure, brain cancer is still rare. In children, it strikes 3.3 in every 100,000 by age 14. But researchers are worried because the incidence seems to be growing at 1.8 percent a year, faster than childhood leukemias – and no one knows why, says NCI epidemiologist Martha Linet.
But the debate continues over whether that increase is real.
“I think the incidence is probably up,” says Dr. Lisa DeAngelis, chairman of the neurology department at Memorial Sloan-Kettering Cancer Center in New York. “It doesn’t seem to be just because of better detection.”
At the Massachusetts General Hospital brain tumor center, however, Dr. John Henson, a neuro-oncologist, disagrees: “I do not think it is going up in older people, and I don’t think it’s going up in children.”
When researchers at the Mayo Clinic reviewed 40 years’ worth of patient records in 1995, they concluded that the apparent increase in brain tumors was attributable to better diagnosis. In older people, for instance, better imaging technologies, like CT and MRI scans, now pick up tumors whose symptoms were once attributed to strokes.
Henson does believe, though, that one type of tumor – brain lymphoma – may be on the rise. This is a rare cancer, he says, but new cases have tripled in the last 20 years, even in people with healthy immune systems. (Most lymphomas affect lymph glands and immune cells thoughout the body, but lymphomas that arise in the brain seem not to affect tissues elsewhere.)
The positive news in all this is that treatments – and survival rates – for primary brain tumors have been improving steadily, especially for kids.
In 1973, says Linet of NCI, the chance that a child under 19 with a malignant brain tumor would survive five years was 54 percent. Today, it’s 73 percent, she says, “a big change.”
And for a tumor called a medulloblastoma, five-year survival is now 80 to 85 percent, says Dr. Roger Packer, chairman of neurology at Children’s National Medical Center in Washington, D.C. A generation ago, he says, it was 30 to 40 percent.
One of those whose chances now look rosier is nine-year old Ellen O’Brien of Melrose. Diagnosed the day after her fifth birthday, Ellen has had three surgeries, chemotherapy and radiation. At one point, in the belief there was no hope for survival, she was sent home for hospice care.
There were some “horrendous” times, like when Ellen was on high-dose steroids, says her mother, Kate. “The child was psychotic. She tried to kill herself.” But Ellen has been fine for two years, and her mom says, “Things are looking great.”
For adults, unfortunately, the news is not quite as encouraging, notwithstanding the miracle of Jordan Fieldman, the young doctor in the Berkshires. Currently, the overall five-year survival for adults with malignant brain tumors is 30 percent, NCI figures show. For very aggressive tumors, the one-year survival is only 50 percent.
Still, there are reasons to hope.
One is that doctors now pay more attention to the quality of life of brain tumor patients, a key issue because treatments historically destroyed so many healthy parts of the brain that even if the tumor was removed, people were left with severe handicaps, such as blindness.
Another is that there have been “tremendous advances in ways of delivering radiation,” says Dr. Jay Loeffler, director of the Northeast Proton Therapy Center at MGH and head of the brain tumor center at Brigham and Women’s Hospital.
One thing that has helped is stereotaxis, a process in which doctors screw a metal frame onto the patient’s skull and then do CT or MRI scans, using markers on the frame as reference points. The result is a three-dimensional map showing where the tumor is. This allows doctors to do “conformal” radiation, that is, to match radiation precisely to the shape of the tumor.
“If the tumor looks like an aligator,” says Loeffler, “we can make the radiation look like that, too.”
Doctors use stereotaxis to guide the radiation, which can either be given in daily doses over six or seven weeks or in a single big dose, a technique called “stereotactic radiosurgery.”
Today, there are even noninvasive stereotactic frames in some hospitals. Instead of screwing a frame to the head, doctors insert a plate into the mouth and use the upper jaw – which doesn’t move – as the fixed point to guide radiation.
Better types of radiation are helping, too. With standard radiation beams, energy is released as it travels through tissues. But at a few centers, doctors now use proton beams, which travel to a specified spot, then release all their energy there, blasting the tumor and minimizing damage to other areas.
Surgery has improved, too. Instead of opening the whole skull to see if a tumor is present, doctors can insert a fine needle, guided by stereotaxis and the scans. Surgeons can also remove some whole tumors this way, too.
And at Brigham and Women’s – so far the only center to do so – doctors now remove some tumors with an MRI scanner in the operating room, getting constant visual clues as they go along.
Other treatments are also promising, including drugs to block new blood vessel growth around tumors, an idea pioneered by Dr. Judah Folkman, director of surgical research at Children’s Hospital in Boston.
Among these anti-angiogenic drugs is the once-reviled sedative thalidomide, which was banned for causing severe birth defects.
Recently, an advisory board to the US Food and Drug Administration recommended its approval for treating a side effect of leprosy. Even if it is approved, however, its for other conditions may be limited.
At the Dana Farber Cancer Institute, Dr. Howard Fine, director of the Center for Neuro-oncology, has been studying both thalidomide and a blood vessel blocker called TNP470, and he says he’s encouraged by the early results.
Fine and others are now trying, in animal studies, to combine blood vessel blockers with gene therapy, a process in which altered viruses that secrete anti-angiogenesis chemicals are injected into tumors.
Other researchers are tinkering with ways to penetrate the “blood-brain barrier” to get more chemotherapy into the brain. This barrier is a unique feature of blood vessels in the brain that makes them less leaky. That protects the brain against toxic chemicals but makes it harder to get useful drugs in.
Still others are working on drugs to make tumor cells evolve from more to less aggressive and drugs to boost immune response.
And then there’s faith in something beyond science.
“When Western medicine gives you zero percent survival, you start exploring other options,” says Jordan Fieldman, who spent six months living with Tibetan monks in India – before his diagnosis. He still meditates every day.
“I was trained in the sciences,” he says. “It would have been easy for me to obey the odds and do what it says in the textbooks. But I had faith I could overcome it.”
“The biggest gift,” he says, “is a far deeper respect and appreciation for life in all its subtle manifestations.”
1. To learn more
An all-day symposium on adult and pediatric brain tumors called “New Frontiers ’97” will take place at the Sheraton Boston Hotel and Towers Saturday from 8 a.m.-6 p.m.
Admission is $ 30 per person, plus $ 25 for each additional family member. Scholarships are available by calling The Brain Tumor Society at 1-800-770-8287 begin_of_the_skype_highlighting 1-800-770-8287 end_of_the_skype_highlighting or 617-783-0340 begin_of_the_skype_highlighting 617-783-0340 end_of_the_skype_highlighting.
You can also sign up via the Internet, at www.tbts.org.
For more information on brain tumors, call:
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]The American Brain Tumor Association, 1-800-886-2282 begin_of_the_skype_highlighting 1-800-886-2282 end_of_the_skype_highlighting.
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The National Brain Tumor Foundation, 1-800-934-CURE begin_of_the_skype_highlighting 1-800-934-CURE end_of_the_skype_highlighting, or 1-800-934-2873 begin_of_the_skype_highlighting 1-800-934-2873 end_of_the_skype_highlighting.
2. Searching for causes
Fears that the incidence of primary brain tumors is rising have led to a search for factors that might account for this, especially in children. Researchers have been able to rule out some hypotheses and are still exploring others.
Exposure to high doses of ionizing radiation, the kind produced by atomic bombs and X-ray treatments (but not diagnostic X-rays), have been linked to brain tumors in both children and adults, says Dr. Martha Linet, an epidemiologist at the National Cancer Institute. But nonionizing radiation, the kind that comes from television sets, power lines, video display terminals and microwave ovens, has not been shown in studies to be a cause, says Linet.
Diet may play a role. Research shows that children born to women who ate lots of fruits and vegetables while they were pregnant are protected against some types of primary brain tumors in childhood.