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Brain Tumor Facts

Facts About Diffuse Intrinsic Pontine Glioma (DIPG)

What is a DIPG?

  • Primary cancerous brain tumor that spreads widely through the brainstem (4, 8)

  • Brainstem controls breathing, heart rate, and nerves and muscles used in seeing, hearing, walking, talking, and eating (8)

  • Brainstem gliomas are highly aggressive brain tumors (7)

  • The worst of pediatric gliomas (15)

  • Usually diagnosed in children between ages 5-9 (6)

  • Pediatric brain tumors are very different biologically than adult counterparts (10)

What are the symptoms?

  • Clumsiness or wobbliness in walking, loss of balance, weakness of a leg and/or arm, double vision, headaches, nausea and vomiting, tilting of the head, facial weakness, unusual sleepiness or change in energy level (4, 8)

  • Symptoms are usually present for 6 months or less at time of diagnosis (4)

What is the cause?

  • Like most childhood brain tumors, the cause is unknown (8)

Are there any treatments options?

  • No surgical options due to diffuse invasion throughout brainstem (4)

  • Surgery would cause severe damage to structures vital for arm and leg movement, eye movement, swallowing, breathing, and even consciousness (4)

  • Participation in clinical trials with innovative therapy is encouraged (4, 9)

  • Radiation is the only real treatment option but benefit is transient (9)

  • Even after radiation, regrowth and progression anticipated within 1 year (9)

  • Chemotherapy benefits unclear, studies show little improvement in survival (4)

  • Steroid treatment almost always indicated for swelling around brainstem (4)

  • Steroid side effects: mood changes, weight gain, fluid retention, glucose instability, high blood pressure, and increased susceptibility to infection (4)

  • Shunts sometimes required due to obstruction of circulating brain fluid (4)

  • After progression, no salvage regimen to extend survival

  • Comfort care is recommended until death (8)

What is the prognosis?

  • Brain tumors are the most common cause of cancer death among children (16)

  • Overall mortality rate of pediatric cancer has decreased in past 20 years, but deaths from brain and spinal cord tumors have increased from 18% to 30% (10)

  • Brainstem tumors are the most dreaded cancers in pediatric oncology due to their dismal prognosis (4)

  • Typically follows an unstoppable course of progression despite treatment, a large majority of die within a year of diagnosis (4)

  • Over 97% die within three years (15)

  • One study showed 37% survival rate at 1 year, 20% at 2 years, and 13% at 3 years, with a median survival of 10 months; only 9 of 119 patients lived longer than 3 years (7)

  • 5-yr survival rate < 20% (13)

  • Over 90% die within 18 months of diagnosis (9)

  • High rate of recurrence or progression (4)

  • Mortality rates remain unchanged in the last decade (12)

How common is it?

  • Brain tumors are the most common pediatric cancer other than leukemia and lymphoma (8)

  • Brain tumors comprise approximately 25% of all pediatric cancers (12)

  • Primary brain tumors constitute the most common solid tumor of childhood (9)

  • Brain tumors are the leading cause of solid tumor cancer death in children (12)

  • Brainstem tumors account for about 10 to 15% of childhood brain tumors (4)

  • About 2/3 of all childhood brain tumors are gliomas (2)

  • Most childhood brainstem gliomas are pontine gliomas (8)

  • 5-10 out of every 100 brain tumors is a DIPG (1)

Why is it so deadly?

  • Many essential areas exist in a relatively small and enclosed space; small amounts of abnormal cell growth cause significant symptoms (2)

  • Due to the tumor location it compresses the vital structures that control life: heartbeat, blood pressure, breathing, swallowing and muscle control and sensation of the limbs (7)

  • Sudden death can result from increased pressure within the brain either from swelling around the tumor or hemorrhage of the tumor (7)

What is life like for a child with a DIPG? (from the stories of DIPG kids)

  • The onset of significant symptoms is quick

  • Immediate hospitalization for lots of tests, blood draws, drugs and surgeries

  • Possible daily sedation which may be necessary for radiation for 6 weeks

  • Diet restrictions

  • Multiple medications to be taken 2-3 times/day

  • Frequent vomiting due to pressure on brainstem

  • Significant weight gain, mood changes, interrupted sleep due to steroids

  • Loss of physical abilities --- starts with loss of muscle control on one side of body, i.e. arm and leg and then progresses into both sides, trunk and head/neck/face until they can no longer walk, stand, sit without support, use their arms, maintain head in upright position, eat, drink, talk and eventually breathe

  • Loss of bowel/bladder control

  • Cognitive abilities are spared --- basically these kids are trapped in a body that won’t cooperate yet their minds are completely intact --- it’s torture!

What about further research?

  • The increase in brain and spinal cord cancer rates in past two decades has been the subject of numerous reports (5)

  • “Considering the rarity, it isn’t economically worthwhile for most drugs to be specifically developed for DIPG” (3)

  • Only two new brain tumor treatments approved in the past 25 years (12)

  • Survival past 12 to 14 months uncommon and new approaches to treating these tumors are urgently needed (14)

  • “It is even suggested that a cure to DIPG might result in a cure for almost every other type of cancer” (3)

  • But……not enough is being done so we need your help!!


1. About Kids Health. (2004). Diagnosing diffuse pontine gliomas.

2. Cohen, K. and Yohay, K. High grade gliomas. The Childhood Brain Tumor Foundation.

3. The Cure Starts Now. Awareness and what you can do.

4. Foer, D and Fisher, P. Brain stem gliomas in childhood. The Childhood Brain Tumor Foundation.

5. Gurney, J., Smith, M., and Bunin, G. CNS and miscellaneous intracranial and intraspinal neoplasms.
National Cancer Institute: SEER Pediatric Monograph.

6. Kieran, M. (2007). Diffuse pontine glioma. UpToDate.

7. Landolfi, J. (2006). Brainstem gliomas. eMedicine from WebMD.

8. National Cancer Institute. (2006). Childhood brain stem glioma treatment: Patient version.

9. National Cancer Institute. (2008). Childhood brain stem glioma treatment: Health professionals version.

10. National Cancer Institute. (2005). Pediatric brain tumors. BenchMarks, 5(2).

11. National Cancer Institute. (2007). A snapshot of pediatric cancers.

12. North American Brain Tumor Coalition. (2008). Brain tumor facts.

13. Pediatric Brain Tumor Foundation. Facts about pediatric brain tumors.

14. St Jude Children’s Research Hospital. Brain tumor: Brain stem glioma.

15. Wagner, S., et al. (2006). Treatment options in childhood pontine gliomas. Journal of Neuro-
Oncology, 79, 281–287.

16. University of California, San Francisco Children’s Hospital. (2006). UCSF establishes pediatric brain
tumor institute.

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