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!!

References:

1. About Kids Health. (2004). Diagnosing diffuse pontine gliomas. http://www.aboutkidshealth.ca.

2. Cohen, K. and Yohay, K. High grade gliomas. The Childhood Brain Tumor Foundation. http://childhoodbraintumor.org

3. The Cure Starts Now. Awareness and what you can do. http://www.thecurestartsnow.org.

4. Foer, D and Fisher, P. Brain stem gliomas in childhood. The Childhood Brain Tumor Foundation. http://www.childhoodbraintumor.org.

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

6. Kieran, M. (2007). Diffuse pontine glioma. UpToDate. http://www.uptodate.com.

7. Landolfi, J. (2006). Brainstem gliomas. eMedicine from WebMD. http://www.emedicine.com.

8. National Cancer Institute. (2006). Childhood brain stem glioma treatment: Patient version. http://www.cancer.gov.

9. National Cancer Institute. (2008). Childhood brain stem glioma treatment: Health professionals version. http://www.cancer.gov.

10. National Cancer Institute. (2005). Pediatric brain tumors. BenchMarks, 5(2). http://www.cancer.gov.

11. National Cancer Institute. (2007). A snapshot of pediatric cancers. http://planning.cancer.gov.

12. North American Brain Tumor Coalition. (2008). Brain tumor facts. http://www.nabraintumor.org.

13. Pediatric Brain Tumor Foundation. Facts about pediatric brain tumors. http://www.pbtfus.org.

14. St Jude Children’s Research Hospital. Brain tumor: Brain stem glioma. http://www.stjude.org.

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. http://www.ucsfhealth.org.