Brain Matters

Wearing pink sparring gloves for boxing and silky pink prizefighting shorts, 11-year-old Jessica Meyer danced down the halls of the radiation therapy clinic to the tune of “Eye of the Tiger” and Katy Perry’s “Roar.” She was there for her “last zap”—her last radiation treatment—and with her mom, Lori, behind the camera, Jessica proved that she can turn anything, even cancer treatment, into a party.

 

It all started on a Sunday afternoon in September 2013 when Jessica was playing around with a new machine at Wal-Mart that checked vision.

She had just made the dance team at her dance studio and was a typical fifth grader, but she realized she couldn’t see out of one eye.

“She was already tiny and thin but had been dropping weight over the summer. She just wasn’t growing at the same rate as her friends. We saw a lot of doctors and suspected a lot of things, but when I got her to the eye doctor, he saw some type of mass,” Lori recalled.

After seeing a pediatric ophthalmologist at the Tennessee Lions Eye Center at the Vanderbilt Eye Institute on the following Tuesday, Jessica had an MRI on Wednesday and was in the hospital Thursday for brain surgery. Through an endonasal approach, a small endoscope was navigated through her nostrils to the base of her skull to sample the brain tumor. The biopsy results diagnosed germinoma, a type of tumor best treated by chemotherapy and radiation.

Jessica had four 21-day cycles of chemotherapy and then 24 radiation treatments at Monroe Carell Jr. Children’s Hospital at Vanderbilt.

­“The hospital was her fifth grade,” Lori said, “But she was awesome. She never cried. She was always happy and had fun and made friends wherever she went.”

 

Tackling brain tumors a team approach

Brain tumors are the second most common childhood cancer, accounting for about 1 in 5 cases. But there are dozens of types of cancer that can occur in the brain, making each individual cancer very rare. About 75 percent of children survive at least five years after diagnosis, but prognosis and treatment plans vary widely depending on the type and location of each tumor.

Children’s Hospital specializes in providing individualized treatment for each patient with an arsenal that includes surgery, chemotherapy and radiation oncology. Some tumors can be removed with surgery and completely cured. Others need chemo and radiation. Others are located in risky areas—the brain stem, which controls breathing and movement, or the optic nerve or ear canal—which makes treatment difficult.

“We have many arrows in our quiver to offer to these patients,” said John “Jay” Wellons III, M.D., MSPH, chief of Pediatric Neurosurgery. “Every parent’s nightmare is to know that the headache your child has had for six weeks is a brain tumor. But the field of pediatric brain tumors has changed vastly in the last 20 years. When my friends say it must be so hard to take out pediatric brain tumors, I actually tell them it’s a joyous thing once done. We get to fundamentally impact these children and families. The days of it being an automatic death sentence, with rare exception, are over.”

Those good outcomes stem from a culture of collaboration among the Pediatric Brain Tumor Program team, which includes neurosurgery, oncology, radiation oncology, radiology, ophthalmology, pathology, nursing, case management, social work and Child Life.

The team meets in person every two weeks for a tumor board meeting to discuss the children under their care. Some cases are new diagnoses, while others are recurring cancer or a puzzling development.

“When I see somebody in my clinic, they’re getting one person’s opinion. When we take their case to the tumor board, they’re getting the consensus of all the specialists that treat pediatric brain tumors at Vanderbilt,” said Robert Naftel, M.D., assistant professor of Neurological Surgery.

“It’s a huge comfort to the patients when they know that we’re going to have a face-to-face meeting with everyone,” added case manager Kathy Jernigan, MSN, RN. “They know we’re going to get in this room and put a bunch of brains together and think about the problem from all angles. They love to know that we’re talking about them.”

 

Treatment options

Most brain tumor patients come to the hospital through the Children’s Hospital Emergency Department. Usually brain tumors disrupt the brain’s fluid system, causing a condition called hydrocephalus. As fluid builds in the head, patients come to the ED with nausea, vomiting or headaches, and then a brain scan shows a suspicious mass. Other patients may experience seizures or vision changes.

The first treatment step is usually surgery, either to remove as much of the tumor as possible, or to retrieve a sample of the tumor for biopsy. Naftel specializes in endonasal surgery, which means going through the nose to reach the brain. He received special fellowship training at the University of Pittsburgh to do endonasal surgery, and collaborates with Justin Turner, M.D., Ph.D., an adult ear, nose and throat surgical specialist at Vanderbilt who has expertise in endonasal approaches for brain tumor removal.

“For about 10 to 15 cases each year, going through the nose is the best way to reach the tumor. You can better reach areas up under the brain that are difficult places to access from a craniotomy (surgical removal of a section of skull bone). It really does give you a direct view of certain areas,” Naftel said.

“Families like the idea that there’s not a scar on their child’s head, but that’s not why we do it. We do it because it provides us the most direct approach to that specific tumor type.”

It’s all about finding the safest route to the tumor, balancing removing as much bad tissue as possible while preserving critical brain function.

“The idea is to be as minimally invasive as you can. To get in and get out in a stealthy way,” said Wellons, who has more than 10 years of experience using endoscopes for brain tumor surgery. “We can go through the nose or eyebrow or make a small incision at the hairline. But at the other end of the spectrum, sometimes we need to make a big incision where we use equally complex approaches that involve removing bone to minimize retraction on the brain. It’s all about what is best for the patient, and being able to offer so many surgical options really feeds into the personalized medicine theme here at Vanderbilt.”

After patients heal from brain surgery, they may need additional treatment to destroy or shrink their tumor.

“We have very good chemotherapy options right now,” said Adam Esbenshade, M.D., MSCI, assistant professor of Pediatrics and a specialist in neuro-oncology. “But to really get to the next level of therapy, we’re trying to get more molecularly targeted and trying to understand what is driving a tumor to act. We’re in the infancy now of this understanding that there are certain mutations driving tumors, but I think we’re on the verge of a lot of breakthroughs.”

Children’s Hospital is part of a worldwide collaboration called the Children’s Oncology Group, which is devoted to childhood cancer research and offers clinical trials to test new therapies.

“In pediatric cancer, we all work together. There are always new trials coming out and we always have people enrolled on those studies,” said Devang Pastakia, M.D., assistant professor of Pediatrics and a specialist in neuro-oncology. “How did medulloblastoma (a brain tumor at the base of the skull) go from 25 to 80 percent survival? It’s because there’s been generations of people who have enrolled in these trials.”

Low-grade tumors (benign) that are able to be fully surgically resected need no other therapy in the vast majority of cases. Those that are not able to be resected typically receive chemotherapy once a week for a year.

“We get very close to the families we take care of,” Pastakia said. “The first thing is we have to get them over the guilt. They all ask, ‘what have I done?’ But from what we can tell brain tumors are a random occurrence. There’s no evidence for why one person gets it over another.”

The Brain Tumor team helps families manage the demands of frequent chemotherapy infusions, radiation treatments and clinic appointments.

“Our mentality is that families go through enough already, and we want to make this as easy as possible. We do this all the time and have the resources to manage school and work and everything this entails,” Pastakia said.

Jessica said with her frequent visits to the hospital, she felt like the staff were part of her family. “They treat you so nicely. It was always scary, but I felt comforted,” she said.

Jessica’s chemotherapy regimen made her very nauseated, so her No. 1 piece of advice is to “take a bunch of Zofran,” a powerful anti-nausea drug. Her No. 2 tidbit is more philosophical:

“Keep a positive attitude during the hard times. Take 3 seconds to complain and then feel positive and happy again.”

Radiation doses for children

After months of chemotherapy, Jessica had radiation therapy, which uses a high-energy, focused X-ray beam to “clean up” any microscopic disease that may have been left

behind. Intensity-modulated radiation therapy (IMRT) is an advanced technology that precisely controls the radiation beams to reduce the exposure of healthy brain tissue and other critical structures, like hearing and vision.

“It uses X-rays, like looking at a broken bone, so there is no pain,” explained Austin Kirschner, M.D., Ph.D., assistant professor of Radiation Oncology. “We give small doses per day to add up to a large dose total. The body heals a little between each treatment, and most children don’t have any symptoms, except some may have hair loss or headache.”

Although all brain tumors are different, the standard course of treatment is once daily, Monday through Friday for about six weeks. Children who are old enough to keep still, usually around age 6 or 7, are fitted with a plastic mesh mask that holds their head in an exact position. Younger patients receive anesthesia. The treatment itself lasts less than 5 minutes, but it can take 20 to 30 minutes to position the child properly.

“It’s critical that the patients don’t move, and we have technology that precisely delivers the radiation to the exact tumor location. We control the dose to maximize the safe level that brain structures can tolerate,” Kirschner said.

Because a child’s brain is still developing, doses of radiation must be carefully considered.

“The long-term side effects are the big worry. These children have their whole life in front of them and we don’t want to cause any permanent damage,” Kirschner said.

“Memory and cognitive processing are the big things that can be harmed. In general, 3 is the minimum age to consider giving radiation to the brain. Younger than that, we’re doing too much harm to the developing brain. Around ages 10-12, the brain is more developed and able to handle the radiation.”

Kirschner said the Children’s Oncology Group also has exciting studies underway in radiation oncology and is anticipating results about radiation doses in germinoma tumors.

“We’re constantly wondering if we could reduce the radiation dose without sacrificing control of the disease. I always wish we could give less.”

 

The big finale

During her treatment, a Child Life specialist suggested that Jessica write down her feelings, but Jessica thought that sounded like homework. Instead, Lori used her smartphone to record videos. The big finale was Jessica’s “Eye of the Tiger” dance down the halls for her final radiation treatment.

“I wanted to look back and see all the cool things I got to do and to educate people on what I was going through,” Jessica said. “It was really tough, but made me look at life differently.”

Over the summer, she worked on her endurance and was able to make the dance team and return to sixth grade at St. Joseph School in Madison full time last fall. She loves math and science, and when she grows up she wants to be an ambassador for the Children’s Miracle Network, a non-profit organization that raises funds for children’s hospitals.

“Her beautiful hair is growing back and she’s getting stronger every day,” Lori said. “I prayed every day that it should have been me not her, but she was a rock star.”