So few children worldwide have the relentless kind of brain cancer that has stricken Alisha Savage that even at Dana-Farber Cancer Institute, the world leader in treating the disease, doctors are still trying to grasp what causes it and why it is so resistant to treatment.
Alisha, just 31⁄2 years old, has atypical teratoid rhabdoid tumor, or AT/RT, a highly aggressive tumor that started in her brain and spread to her spine. After a year of treatment in her native Ireland, Alisha’s parents brought her to Boston this month to see experts at Dana-Farber.
Dr. Mark Kieran, clinical director of the Brain Tumor Center at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, called AT/RT “exceedingly rare” — so rare that Dana-Farber sees fewer than half a dozen patients a year. The disease’s rarity also makes it harder to study.
“They grow very rapidly,” Kieran said of the tumors. “They tend to spread quickly, so you’re not treating one tumor, you’re treating many.”
And the tumors tend to resist even intensive chemotherapy and surgery.
The disease starts early in children — it can even be seen at birth. Avalanna Routh of Merrimac, who captured hearts nationwide as “Mrs. Justin Bieber” when a mock wedding thrown by hospital staff led to a Valentine’s Day date with the Canadian pop star, was diagnosed with AT/RT at 9 months old. She died of the disease in 2012 at age 6.
The survival rate for those treated is about 50 percent, but Dana-Farber researchers are testing new, targeted treatments that might be more promising.
“We’re doing some new things,” Kieran said. “Whether they will work remains to be seen.”
Little Alisha, whose family is from Cork, was getting treatment in Dublin for a year before parents Michelle Long and John Savage felt like they were out of options and brought her to Boston to have access to the newest treatments.
“The longer she can survive it, the more research that can be done,” Savage said. “At home there was nothing.”
Alisha has already endured a series of surgeries, chemotherapy and radiation treatment. She has scars on her head, one in the front from the port used to give her chemotherapy, and another in the back where doctors in Ireland removed as much of her tumor as they could.
Her parents don’t know how long she will need to stay in Boston for treatment, or how much it will cost, though they know the numbers will be staggering.
Researchers say they still have much work to do in identifying treatments for kids with AT/RT without damaging healthy parts of their bodies.
“This particular cancer is more difficult than some of the other ones,” said Laura MacConaill, who is hosting Alisha’s family in her Hanover home and also is a scientist at Dana-Farber who studied AT/RT. “To be able to identify the genomic underpinnings of this disease is very important.”
