Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatmentPrognosisCoping
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
Prognosis
Coping
Diffuse intrinsic pontine glioma (DIPG) is a rare, aggressive, and deadly type of brain cancer that primarily affects children under the age of 10.
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The tumor grows in thebrainstem, which sits at the back of the neck where the brain meets the spinal cord. It’s made up of glial cells, which makes it a type ofglioma. Glial cells act as supportive cells around neurons.
With DIPG, glial cells grow out of control, eventually squeezing and damaging the healthy brain tissue.This resulting brain damage leads to serious and life-threatening consequences.
How Rare Is DIPG?Only about 200 to 300 new DIPG cases are diagnosed each year in the U.S.It most often strikes between the ages of 6 and 9 and is slightly more common in boys than girls. DIPG is deadly—many of those with DIPG survive for less than one year after diagnosis.And 90% will die within two years of diagnosis.
How Rare Is DIPG?
Only about 200 to 300 new DIPG cases are diagnosed each year in the U.S.It most often strikes between the ages of 6 and 9 and is slightly more common in boys than girls. DIPG is deadly—many of those with DIPG survive for less than one year after diagnosis.And 90% will die within two years of diagnosis.
DIPG Symptoms
DIPG is a fast-growing tumor, with symptoms typically appearing suddenly and quickly becoming dramatically worse. These tumors occur in thepons, an area of the brainstem that is responsible for many of the body’s essential functions, such as breathing, blood pressure control, and eye movement.
Common DIPG symptoms include:
Disease Progression and End of Life
The effects of DIPG can become life-threatening in a matter of weeks to months.
Some of the most common symptoms in the last three months of life are:
DIPG and End of LifeIn one study, 17% of children with DIPG needed to be placed onventilatorsso they could breathe, and nearly half of the study participants received drugs for pain and agitation.
DIPG and End of Life
In one study, 17% of children with DIPG needed to be placed onventilatorsso they could breathe, and nearly half of the study participants received drugs for pain and agitation.
These problems become progressively worse as time goes on. In the last days of life, children with DIPG have increasing problems with breathing and consciousness.
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Recent research shows that 80% of DIPG tumors have amutationin the gene that codes for a protein called histone H3.Histones are proteins that allowDNAto wrap around a chromosome and fit into the nucleus of a cell.
Other research links about 25% of DIPG cases to mutations in a gene called ACVR1.
Early childhood is a prime time for brain development and, that may help explain why DIPG affects young children so disproportionately.
If your child has symptoms of DIPG, their doctor will ask you about the symptoms that your child is experiencing and performneurological testing, looking at balance and movement in the arms and legs.
DIPG is usually diagnosed with imaging tests.
Your child’s doctor may order:
Biopsy
If your child’s imaging test is inconclusive, their doctor may recommend a biopsy to make a definitive diagnosis. However, a biopsy is often not done due to the location of the tumor. A biopsy may put the person at risk for additional problems.
DIPG tumors are mainly treated withradiation therapy, which uses specialized high-energy X-rays to prevent cancer cells from dividing. This is generally used in children older than 3.The radiation is delivered five days a week for about six weeks.
If the tumor grows back, more radiation can be given to reduce symptoms and extend a child’s life.
It’s important to note that there is no cure for DIPG, and therapies used to treat other types of tumors are often not effective.
Given the fact that DIPG doesn’t produce a solid mass, but instead creeps into the nerve fibers of the pons, standard neurosurgery isn’t a viable option.The delicate area of the brain in which these tumors are found also makes surgery especially dangerous.
Many chemotherapy drugs can’t cross the blood-brain barrier efficiently enough to have a therapeutic effect on the tumor.
Palliative and Hospice Care
As the end of life nears, many families look intopalliative careor hospice care to help them grapple with tough issues and give their children the most comfortable end-of-life experience.
A palliative or hospice care team can help you decide what measures to take if your child:
All these end-of-life symptoms are extremely difficult for any parent to witness. A hospice care team can guide you on what to expect, tell you if your child is feeling pain or distress, and advise you on what comfort measures to take—for all involved.
Advanced Drug Research
For over 40 years, there’s been little advancement in DIPG treatments. Some progress is now being made. More testing and trials are needed, but some promising advances include:
Typically, children with DIPG survive an average of eight to 10 months after diagnosis, although every child is different. About 10% of kids will survive two years, and 2% will live five years.
DIPG and Life ExpectancyWhen compared to others with DIPG, those who tend to have a longer life expectancy are those who:Are younger than three years old or older than 10 years old at the time of symptom onsetExperience symptoms longer before being diagnosedHave the H3.1K27M (a Histone 3) mutationReceive EGFR inhibitors (drugs that stop or slow cell growth) at diagnosis
DIPG and Life Expectancy
When compared to others with DIPG, those who tend to have a longer life expectancy are those who:Are younger than three years old or older than 10 years old at the time of symptom onsetExperience symptoms longer before being diagnosedHave the H3.1K27M (a Histone 3) mutationReceive EGFR inhibitors (drugs that stop or slow cell growth) at diagnosis
When compared to others with DIPG, those who tend to have a longer life expectancy are those who:
Here are some suggestions:
A Word From Verywell
DIPG is a deadly and devastating disease. But there’s reason for hope. Targeted therapies and more effective drug-delivery systems are in development and showing promise. And the survival rate is inching up, from a 1% five-year survival rate to a 2% one today. That’s a small but significant jump to any family with a child with DIPG.
21 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Weill Cornell Brain and Spine Center.Diffuse intrinsic pontine glioma (DIPG).Cincinnati Children’s.Diffuse intrinsic pontine glioma (DIPG).Warren KE.Diffuse intrinsic pontine glioma: poised for progress.Front Oncol. 2012;2:205. doi:10.3389/fonc.2012.00205Chen J, Lin Z, Barrett L, Dai L, Qin Z.Identification of new therapeutic targets and natural compounds against diffuse intrinsic pontine glioma (DIPG).Bioorg Chem. 2020 Jun;99:103847. doi:10.1016/j.bioorg.2020.103847Veldhuijzen van Zanten, SE, van Meerwijk, et al.Palliative and end-of-life care for children with diffuse intrinsic pontine glioma: Results from a London cohort study and international survey.Neuro-oncology, 2015;18(4), 582–588. doi:10.1093/neuonc/nov250St. Jude Children’s Research Hospital.Diffuse intrinsic pontine glioma (DIPG).Pellot JE, De Jesus, O.Diffuse intrinsic pontine glioma. StatPearls.Hasan F, Weingarten K, Rapoport A,et al.End-of-life care of children with diffuse intrinsic pontine glioma.J Neurooncol2018;138,147–153. doi:10.1007/s11060-018-2781-0National Cancer Institute.Studies identify potential treatment strategies for pediatric DIPG brain tumors.National Human Genome Research Institute.Histone.Carvalho D, Taylor KR, Olaciregui NG, et al.ALK2 inhibitors display beneficial effects in preclinical models ofACVR1mutant diffuse intrinsic pontine glioma.Commun Biol. 2019;2:156. doi:10.1038/s42003-019-0420-8Dana Farber Cancer Institute/Boston Children’s Cancer and Blood Disorders Center.Childhood diffuse intrinsic pontine glioma (DIPG).St. Jude Children’s Research Hospital.Diffuse intrinsic pontine glioma (DIPG).DIPG.org.Radiation therapy for DIPG.Boston Chidren’s Hospital.Overturning dogma to open the black box of DIPG.Weill Cornell Brain and Spine Center.Diagnosing and treating DIPG.DIPG.org.Emerging treatments for DIPG.American Cancer Society.How immunotherapy is used to treat cancer.DIPG.org.DIPG survival rate and prognosis.Hoffman LM, Veldhuijzen van Zanten SEM, et al.Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): A collaborative report from the International and European Society for Pediatric Oncology DIPG Registries.Journal of Clinical Oncology.2018;36:19,1963-1972. doi:10.1200/JCO.2017.75.9308DIPG Registry.End of life care.
21 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Weill Cornell Brain and Spine Center.Diffuse intrinsic pontine glioma (DIPG).Cincinnati Children’s.Diffuse intrinsic pontine glioma (DIPG).Warren KE.Diffuse intrinsic pontine glioma: poised for progress.Front Oncol. 2012;2:205. doi:10.3389/fonc.2012.00205Chen J, Lin Z, Barrett L, Dai L, Qin Z.Identification of new therapeutic targets and natural compounds against diffuse intrinsic pontine glioma (DIPG).Bioorg Chem. 2020 Jun;99:103847. doi:10.1016/j.bioorg.2020.103847Veldhuijzen van Zanten, SE, van Meerwijk, et al.Palliative and end-of-life care for children with diffuse intrinsic pontine glioma: Results from a London cohort study and international survey.Neuro-oncology, 2015;18(4), 582–588. doi:10.1093/neuonc/nov250St. Jude Children’s Research Hospital.Diffuse intrinsic pontine glioma (DIPG).Pellot JE, De Jesus, O.Diffuse intrinsic pontine glioma. StatPearls.Hasan F, Weingarten K, Rapoport A,et al.End-of-life care of children with diffuse intrinsic pontine glioma.J Neurooncol2018;138,147–153. doi:10.1007/s11060-018-2781-0National Cancer Institute.Studies identify potential treatment strategies for pediatric DIPG brain tumors.National Human Genome Research Institute.Histone.Carvalho D, Taylor KR, Olaciregui NG, et al.ALK2 inhibitors display beneficial effects in preclinical models ofACVR1mutant diffuse intrinsic pontine glioma.Commun Biol. 2019;2:156. doi:10.1038/s42003-019-0420-8Dana Farber Cancer Institute/Boston Children’s Cancer and Blood Disorders Center.Childhood diffuse intrinsic pontine glioma (DIPG).St. Jude Children’s Research Hospital.Diffuse intrinsic pontine glioma (DIPG).DIPG.org.Radiation therapy for DIPG.Boston Chidren’s Hospital.Overturning dogma to open the black box of DIPG.Weill Cornell Brain and Spine Center.Diagnosing and treating DIPG.DIPG.org.Emerging treatments for DIPG.American Cancer Society.How immunotherapy is used to treat cancer.DIPG.org.DIPG survival rate and prognosis.Hoffman LM, Veldhuijzen van Zanten SEM, et al.Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): A collaborative report from the International and European Society for Pediatric Oncology DIPG Registries.Journal of Clinical Oncology.2018;36:19,1963-1972. doi:10.1200/JCO.2017.75.9308DIPG Registry.End of life care.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Weill Cornell Brain and Spine Center.Diffuse intrinsic pontine glioma (DIPG).Cincinnati Children’s.Diffuse intrinsic pontine glioma (DIPG).Warren KE.Diffuse intrinsic pontine glioma: poised for progress.Front Oncol. 2012;2:205. doi:10.3389/fonc.2012.00205Chen J, Lin Z, Barrett L, Dai L, Qin Z.Identification of new therapeutic targets and natural compounds against diffuse intrinsic pontine glioma (DIPG).Bioorg Chem. 2020 Jun;99:103847. doi:10.1016/j.bioorg.2020.103847Veldhuijzen van Zanten, SE, van Meerwijk, et al.Palliative and end-of-life care for children with diffuse intrinsic pontine glioma: Results from a London cohort study and international survey.Neuro-oncology, 2015;18(4), 582–588. doi:10.1093/neuonc/nov250St. Jude Children’s Research Hospital.Diffuse intrinsic pontine glioma (DIPG).Pellot JE, De Jesus, O.Diffuse intrinsic pontine glioma. StatPearls.Hasan F, Weingarten K, Rapoport A,et al.End-of-life care of children with diffuse intrinsic pontine glioma.J Neurooncol2018;138,147–153. doi:10.1007/s11060-018-2781-0National Cancer Institute.Studies identify potential treatment strategies for pediatric DIPG brain tumors.National Human Genome Research Institute.Histone.Carvalho D, Taylor KR, Olaciregui NG, et al.ALK2 inhibitors display beneficial effects in preclinical models ofACVR1mutant diffuse intrinsic pontine glioma.Commun Biol. 2019;2:156. doi:10.1038/s42003-019-0420-8Dana Farber Cancer Institute/Boston Children’s Cancer and Blood Disorders Center.Childhood diffuse intrinsic pontine glioma (DIPG).St. Jude Children’s Research Hospital.Diffuse intrinsic pontine glioma (DIPG).DIPG.org.Radiation therapy for DIPG.Boston Chidren’s Hospital.Overturning dogma to open the black box of DIPG.Weill Cornell Brain and Spine Center.Diagnosing and treating DIPG.DIPG.org.Emerging treatments for DIPG.American Cancer Society.How immunotherapy is used to treat cancer.DIPG.org.DIPG survival rate and prognosis.Hoffman LM, Veldhuijzen van Zanten SEM, et al.Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): A collaborative report from the International and European Society for Pediatric Oncology DIPG Registries.Journal of Clinical Oncology.2018;36:19,1963-1972. doi:10.1200/JCO.2017.75.9308DIPG Registry.End of life care.
Weill Cornell Brain and Spine Center.Diffuse intrinsic pontine glioma (DIPG).
Cincinnati Children’s.Diffuse intrinsic pontine glioma (DIPG).
Warren KE.Diffuse intrinsic pontine glioma: poised for progress.Front Oncol. 2012;2:205. doi:10.3389/fonc.2012.00205
Chen J, Lin Z, Barrett L, Dai L, Qin Z.Identification of new therapeutic targets and natural compounds against diffuse intrinsic pontine glioma (DIPG).Bioorg Chem. 2020 Jun;99:103847. doi:10.1016/j.bioorg.2020.103847
Veldhuijzen van Zanten, SE, van Meerwijk, et al.Palliative and end-of-life care for children with diffuse intrinsic pontine glioma: Results from a London cohort study and international survey.Neuro-oncology, 2015;18(4), 582–588. doi:10.1093/neuonc/nov250
St. Jude Children’s Research Hospital.Diffuse intrinsic pontine glioma (DIPG).
Pellot JE, De Jesus, O.Diffuse intrinsic pontine glioma. StatPearls.
Hasan F, Weingarten K, Rapoport A,et al.End-of-life care of children with diffuse intrinsic pontine glioma.J Neurooncol2018;138,147–153. doi:10.1007/s11060-018-2781-0
National Cancer Institute.Studies identify potential treatment strategies for pediatric DIPG brain tumors.
National Human Genome Research Institute.Histone.
Carvalho D, Taylor KR, Olaciregui NG, et al.ALK2 inhibitors display beneficial effects in preclinical models ofACVR1mutant diffuse intrinsic pontine glioma.Commun Biol. 2019;2:156. doi:10.1038/s42003-019-0420-8
Dana Farber Cancer Institute/Boston Children’s Cancer and Blood Disorders Center.Childhood diffuse intrinsic pontine glioma (DIPG).
DIPG.org.Radiation therapy for DIPG.
Boston Chidren’s Hospital.Overturning dogma to open the black box of DIPG.
Weill Cornell Brain and Spine Center.Diagnosing and treating DIPG.
DIPG.org.Emerging treatments for DIPG.
American Cancer Society.How immunotherapy is used to treat cancer.
DIPG.org.DIPG survival rate and prognosis.
Hoffman LM, Veldhuijzen van Zanten SEM, et al.Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): A collaborative report from the International and European Society for Pediatric Oncology DIPG Registries.Journal of Clinical Oncology.2018;36:19,1963-1972. doi:10.1200/JCO.2017.75.9308
DIPG Registry.End of life care.
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