A new approach to old ideas about diffuse intrinsic pontine glioma

Hilary Olson had no reason to suspect that her daughter Hailey might have a brain tumor.

“Her smile was starting to droop a little, and one of her eyes was a little jumpy,” says the 6-year-old’s mother. “We took her to see a neurologist, and he thought she might have pinched a nerve.

“But when he sent us to Boston Children’s Hospital for an MRI,” she continues, “the radiologists sent us straight down to the emergency room.”

Hailey’s diagnosis came as a huge jolt: a rare, almost always fatal tumor called diffuse intrinsic pontine glioma (DIPG). “The doctors were shocked by the size of the tumor,” Hilary recalls.

Hailey Olson with Mark Kieran, MD, PhD, and Susan Chi, MD

DIPGs are so fatal because doctors haven’t had any success at treating them — and in fact haven’t known where to start. The problem has to do with where they occur. DIPGs nestle among the nerves in a portion of the brain stem called the pons, which controls our breathing, blood pressure and heart rate.

“For 40 years, we didn’t have the surgical techniques to safely take a biopsy of a DIPG,” says Mark Kieran, MD, PhD, director of the Brain Tumor Program at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, a partnership between Dana-Farber and Boston Children’s focused on treating children with cancer.

“In fact, it’s still part of the dogma taught to every oncologist — ‘Don’t biopsy brain stem gliomas’ — because of fears about the risk of severe or fatal damage. And because we couldn’t biopsy DIPGs, we couldn’t study them to learn what makes them tick.”

Looking to turn that dogma on its head, Kieran has launched a DIPG clinical trial that takes advantage of the last 40 years’ worth of advances in neurosurgery and in our understanding of the biology of cancer to target and personalize DIPG treatment. Hailey is the first patient.

“At first the doctors told us that Hailey’s tumor was inoperable, but in that first week we got the call telling us about this new clinical trial,” says Hilary. “It had been approved two days after Hailey was diagnosed.”

The trial — currently open only at Dana-Farber/Boston Children’s, though another 19 centers will soon start participating — brings the hope that, in the future, more children will be able to survive this largely mysterious tumor. Kieran hopes to recruit between 25 and 100 children.

“For the first time, we should be able to give children with DIPG like Hailey personalized treatment options based on the makeup of their individual tumor,” Kieran says. “We have the opportunity to look within DIPG and understand why it differs so greatly from other tumors, which will help us map out better strategies for the future.”

This story first appeared on Boston Children’s Hospital’s Thriving blog. Read more about Dr. Kieran’s research into DIPG in the full story, From boos to hope: Challenging the dogma about deadly brain stem gliomas. For more about treating pediatric brain tumors, watch Dr. Kieran’s webinar on the topic, which appears on the American Brain Tumor Association website.

10 thoughts on “A new approach to old ideas about diffuse intrinsic pontine glioma”

  1. My nephew was just disnosed with diffuse pontine instrinsic glioma. If you have any information it would please let me know. The family is willing to go anywhere and do Anthony.

  2. I have been an advocate for children with DIPG for quite some time, and I have seen them go through trial after trail. Since DIPG is always fatal, it is wonderful to see some new research going into this to give the patients hope!

  3. Hello. My daughter Delaney was diagnosed with DIPG in October of 2011. She had 2 biopsies done along with a VP shunt. She had the 6 weeks of radiation and had an 80% reduction. Today she is still stable and has her next MRI in April. I believe her hospital still has the sample if you want to view it. We are on edge with fear that it could be any time that it progresses. I hope and pray that a cure or help is around the corner for so many children and their families being affected by this. Thank you.

    • Dear Heath,

      I read your entry and was filled with hope. I am so happy to hear that Delaney is fighting the fight and I hope that her April MRI yielded great results. Please do let me know how Delaney is faring. My nephew Peter was diagnosed with DIPG in January 2013 and he completed is radiation and currently on chemo, along with some additional alternative therapies. His first to MRIs after radiation showed reduction in the tumour and we are hoping his next one at the end of September will show the same. God bless you and your family and all those who have to deal with this terrible ordeal.

    • Great to know about Delaney. Where did you get the Biopsy done? Did you know the risks associated with biopsy that time? Did they sound very confident about doing it? My 4.5 yr old has DIPG and her radiation therapy starts 9/11/2014

  4. I think it is aawesome that someone has finally decided to delve into this devastating tumor. Our son was diagnosed with dipg in 1987 and we were told his prognosis on life was 1 year or less. We were also told that this tumor had been researched for 20 years and still there had been no results as to how to attack it other than radiation and chemo if we chose to go that route. Sadly our son lost his battle almost a year after he was diagnosed. I am so happy that there has been some recent development into possible treating this with success. May God guide all of the doctors with their hands and brains as to eradicating this horrible destructive tumor.

    • My son is 15 and we were just told he has DIPG also. I have cried my eyes out for days, but its time to find help now. Has anyone survived this ever?

  5. There have been many advacements .I prey and hope there is a cure soon. My Son name is Josiah who is 5 YRS OLD. he was. diagnosed april 22nd,2013. He went thru 6 weeks of rdiation and 6 week clinical study known as SAHA. Thru 3 MRI s it showed it . Shrank. Over 85% . the tumour shrnk slightly the on the 3rd scan..and a week later ..symtoms represented themselves. THE 4TH MRI SHOWED NO GROWTH..but even a microsopic change can trigger the symtoms…u will see the smptoms before u c the growth on the MRI. doctors have declared Josiah in progressive state..he is still doing well considering. ARNOLD PALMER IS WERE HE RECIEVES HIS TREATMENT. you can follow. Josiah on Josiahs journey on fb.

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