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. 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.