June 16, 2025 • 10 mins
Today, Victoria W, a student at Detroit Country Day School, dives deep into diffuse intrinsic pontine glioma, or DIPG, a rare and aggressive form of brain cancer that primarily affects children. As she explores its causes and symptoms, she highlights the alarming fact that only about 2% of diagnosed children survive past five years. Victoria discusses the personal story of Erin, a brave young girl who faced DIPG and became an advocate for awareness and research funding before her untimely passing. Victoria covers the latest research, including potential links to the hedgehog signaling pathway, and innovative treatment options like microbeam radiation therapy, which promises to spare healthy brain tissue while targeting cancer cells more effectively. Join her as she unravels the complexities of DIPG and looks toward a future where a cure might finally be within reach.
Mark as Played
Transcript
Episode Transcript
Available transcripts are automatically generated. Complete accuracy is not guaranteed.
(00:08):
Hello, I'm Victoria Wong andI'm a junior at Detroit Country Day
School.
I'm currently taking APBiology and I have always been fascinated
by neuroscience and everythingrelated to the brain.
A few years ago, I was doingresearch about different neurological
diseases when I stumbled upondiffuse intrinsic pontine gliomark
aggressive form of brain cancer.
Ever since I learned aboutdipg, I have been constantly checking
(00:31):
on the research beingconducted about it and waiting for
updates about a cure.
In this podcast, we will diveinto the causes and symptoms of diffuse
intrinsic pontine glioma andthe potential cures that scientists
have been developing over thepast few years.
So without further ado, let'sbegin our study of DIPG.
At six years old, Erin was anenergetic child.
(00:53):
However, at 11 years old, shebegan to experience severe headaches,
nausea and vomiting.
After getting a CT scan, shewas diagnosed with dipg, a rare brain
tumor that develops in thebrainstem and affects the nervous
system.
She was given radiation,chemotherapy and surgery.
Erin survived past herprognosis and began campaigning to
raise awareness and increasefunding for childhood cancer.
(01:17):
However, in 2014, Erindeteriorated very quickly with headaches,
poor balance and seizures.
She was hospitalized and lostthe ability to speak, eventually
passing away.
Afterwards, Erin donated hertumor to researchers, hoping that
the research would helpdevelop a cure for dipg.
Diffuse intrinsic Pontineglioma is a fast growing malignant
(01:38):
brain tumor that begins in thePONS region of the brain.
The PONS is part of thebrainstem and plays a vital role
in controlling bodilyfunctions such as breathing, heart
rate, blood pressure and manyother muscles throughout the body.
Because dupont plays such amajor role in controlling the body,
the symptoms of DIPG becomenoticeable quickly and the latency
period is very short.
(01:58):
DIPG is also different fromother high grade gliomas because
it is very diffuse.
Because DIPG spreads to thebrainstem, cerebellum and cerebrum,
it is especially hard to treatand extremely deadly.
Every year, around 300 kidsare diagnosed with the IPG in the
US while DIPG can occur inyounger children and teens, most
of the diagnosed children arebetween the ages of 5 and 10 years
(02:22):
old.
Unfortunately, as of now, thesurvival rate for children with DIPG
is very low.
Only around 2% of the childrenlive past five years after diagnosis
and long term survival is very rare.
Ultimately, the average time achild with DIPG survives after diagnosis
is 8 to 11 months.
(02:42):
Now what exactly causes DIPG?
DIPG is a glioma and gliomasare caused by changes in the cell
cycle of glial cells.
Glial cells protect thebrain's nerve cells and play a vital
role in the brain, making thisform of cancer particularly harmful.
In general, changes to thecell cycle, such as a non functionally
regulating system, can causethe cells to divide uncontrollably.
(03:05):
For example, mutated tumorsuppressor genes such as p53, a gene
responsible for apoptosis, maycause the cell to growth arrest and
cell death.
Oncogenes, which are mutatedproto oncogenes, can cause continuous
cell growth and division.
In the end, both the mutatedtumor suppressor gene and the oncogene
can cause cancer.
(03:26):
Although scientists are awareof these mutations, the exact causes
for the changes in the glialcells is unknown.
While scientists have notquite determined the exact cause
for dipg, more research iscurrently being conducted.
In a study by Michelle Mongyand other scientists, DIPG has been
linked to the dysregulation ofpostnatal development during middle
(03:47):
childhood and is connected toan active hedgehog signaling pathway.
The hedgehog signaling pathwayis important in vertebrate embryonic
development and it controlsthe formation and morphogenesis of
an organism.
Monge's experiment studiedcell populations with nestin, an
intermediate filament proteinin the ventral brainstem.
In the pons, during infancy,cells with NESTIN are present in
(04:10):
the ventral brainstem structure.
These cells decrease by theage of two.
However, in the human pons,the density of these cells peaks
around six years old, whichcorresponds to the incidence of dipg.
The hedgehog pathway is alsopart of the neural precursor cell
populations.
Scientists use betagalactosidase to determine the presence
(04:32):
of an active hedgehog pathway.
In the ventral ponds.
The beta galactosidasepositive cells were positive for
olig 2, a transcription factorknown to be regulated in the embryonic
spinal cord by the sonichedgehog gene and SOX2, a marker
of primitive cell types.
These cells increaseddramatically in middle childhood.
Thus, the hedgehog signalingpathway plays a role in the development
(04:54):
of dipg.
To ensure that the hedgehogsignaling pathway was causing the
cancer, the scientists usedthe sonic hedgehog protein ligand
which activates the hedgehogsignaling pathway and and they also
used a hedgehog antagonistwhich represses the pathway.
They then measured the redfluorescent protein produced from
the hedgehog pathway.
(05:14):
When the pathway was exposedto the hedgehog antagonist, the red
fluorescent protein decreasedby 67%.
When the sonic hedgehog ligandwas added, the red fluorescent protein
increased by 200%, suggestingthat the hedgehog pathway was indeed
involved.
Even though scientists found acorrelation between DIPG and an active
hedgehog pathway.
(05:35):
The dysregulation of thehedgehog pathway pathway doesn't
necessarily cause dipg.
This is evident since somediseases have inactive hedgehog pathways,
but the people do not develop dipg.
But a constantly activehedgehog signaling pathway is associated
with the proliferation of DIPG cells.
Now, the symptoms for DIPG arelargely dependent on where the tumor
(05:57):
forms, the size of the tumor,how fast the tumor grows, and the
child's age.
However, the symptoms developquickly and include trouble with
eye movements, visionproblems, problems with talking,
chewing and swallowing,drooping on one side of the face,
headaches, nausea andvomiting, weakness in the arms and
legs, loss of balance, troublewalking, changes in behavior and
trouble learning in school.
(06:19):
There are also many differentmethods for diagnosis.
A magnetic resonance imaging,also known as an mri, creates pictures
of areas inside the brain.
When gadolinium, a rare earthmetal used for MRIs, is injected
into a vein, it collectsaround cancer cells, making the cancer
more prominent in theOccasionally, a biopsy, specifically
(06:40):
a stereotactic biopsy thatuses imaging techniques to find and
remove the tumor tissue can be performed.
However, because of thelocation of the tumor, it isn't always
the best option.
Finally, immunohistochemistrycan also be done.
It uses antibodies to checkfor antigens in tissues and these
antigens are often used todetermine if the cancer is present.
(07:02):
Currently, there are no curesfor dipg, but there are treatments
that can be used to extend thepatient's lives.
Such treatments includeradiation therapy, chemotherapy and
surgery.
Radiation therapy uses highenergy X rays and other forms of
radiation to kill cancer cells.
Since this causes changes inbrain tissue, it can cause damage
to the brain, affecting growthand brain development.
(07:25):
Chemotherapy is another optionthat uses drugs to slow or stop the
growth of cancer cells.
It can either stop the cellsfrom dividing or kill them altogether.
Finally, surgery can be usedto place a shunt.
This is often needed forchildren with DIPG who have fluid
around their brain or spinal cord.
The shunt carries extra fluidaway from the brain, which ultimately
(07:46):
decreases the fluid and thepressure on the brain and spinal
cord.
Recently, scientists have alsofound several promising methods to
combat the cancer.
I'm going to talk about onespecific method that Ellum Smith
and other scientists have beenstudying, Synctron microbeam radiation
therapy.
Recently, scientists haveconsidered this microbeam radiation
(08:07):
therapy as a potentialalternative to the conventional radiation
therapy being used to treat dipg.
Microbeam radiation therapyhas been especially promising because
it spares normal brain tissuesby using spatial dose distribution.
This reduces the damage causedto the brain by conventional radiation
therapy and it slows down theprogression of the tumor.
(08:28):
Still, scientists have testedthe microbeam radiotherapy for efficiency
by examining apoptosis and theprogression of the cell cycle in
cancer cells that have beenexposed to it.
They found that in cell lineswith an active p53, the gene that
creates the proteinresponsible for apoptosis, apoptosis
of the cancer cells increases significantly.
(08:48):
Additionally, those cells werefound to have more cells in the sub
G0 phase, which is also knownas apoptosis, and decreased number
of cells in the G0, G1, G2 andM phases of the cell cycle.
Thus, apoptosis increased andthe cell cycle decreased after the
microbeam radiation.
(09:08):
For cell lines without afunctioning p53 gene, there was a
decrease in the cells in theG0 and G1 phase.
However, there was an increasein the sub G0 and G2 and M phases.
This accumulation of cells inthe G2 and M phases suggests that
there was a prolonged mitosis,so the DNA that was reduplicated
(09:29):
was not separated correctlyand accumulated in the wrong phase.
Since the DNA was notseparated correctly, polyploidy,
which occurs when an organismhas the wrong number of chromosomes,
occurred.
Ultimately, these polyploidcells were reproductively dead, resulting
in very little cell duplication.
Thus, because of theseresults, microbeam radiotherapy proves
(09:49):
to be an alternativeradiotherapy treatment for patients
with dipg because it is moredamaging for cancer cells and less
damaging for normal tissues.
While DIPG is still underresearched, the current research
being conducted by scientistshave led to major breakthroughs about
the causes and potential curesfor the cancer.
There is still a lot of workthat must be done, but a future cure
(10:10):
for DIPG is currently in the works.
Thank you for listening.
Hello, I'm Victoria Wong andI'm a junior at Detroit Country Day
School.
I'm currently taking APBiology and I have always been fascinated
by neuroscience and everythingrelated to the brain.
A few years ago, I was doingresearch about different neurological
diseases when I stumbled upondiffuse intrinsic pontine gliomark
aggressive form of brain cancer.
Ever since I learned aboutdipg, I have been constantly checking
(00:31):
on the research beingconducted about it and waiting for
updates about a cure.
In this podcast, we will diveinto the causes and symptoms of diffuse
intrinsic pontine glioma andthe potential cures that scientists
have been developing over thepast few years.
So without further ado, let'sbegin our study of DIPG.
At six years old, Erin was anenergetic child.
(00:53):
However, at 11 years old, shebegan to experience severe headaches,
nausea and vomiting.
After getting a CT scan, shewas diagnosed with dipg, a rare brain
tumor that develops in thebrainstem and affects the nervous
system.
She was given radiation,chemotherapy and surgery.
Erin survived past herprognosis and began campaigning to
raise awareness and increasefunding for childhood cancer.
(01:17):
However, in 2014, Erindeteriorated very quickly with headaches,
poor balance and seizures.
She was hospitalized and lostthe ability to speak, eventually
passing away.
Afterwards, Erin donated hertumor to researchers, hoping that
the research would helpdevelop a cure for dipg.
Diffuse intrinsic Pontineglioma is a fast growing malignant
(01:38):
brain tumor that begins in thePONS region of the brain.
The PONS is part of thebrainstem and plays a vital role
in controlling bodilyfunctions such as breathing, heart
rate, blood pressure and manyother muscles throughout the body.
Because dupont plays such amajor role in controlling the body,
the symptoms of DIPG becomenoticeable quickly and the latency
period is very short.
(01:58):
DIPG is also different fromother high grade gliomas because
it is very diffuse.
Because DIPG spreads to thebrainstem, cerebellum and cerebrum,
it is especially hard to treatand extremely deadly.
Every year, around 300 kidsare diagnosed with the IPG in the
US while DIPG can occur inyounger children and teens, most
of the diagnosed children arebetween the ages of 5 and 10 years
(02:22):
old.
Unfortunately, as of now, thesurvival rate for children with DIPG
is very low.
Only around 2% of the childrenlive past five years after diagnosis
and long term survival is very rare.
Ultimately, the average time achild with DIPG survives after diagnosis
is 8 to 11 months.
(02:42):
Now what exactly causes DIPG?
DIPG is a glioma and gliomasare caused by changes in the cell
cycle of glial cells.
Glial cells protect thebrain's nerve cells and play a vital
role in the brain, making thisform of cancer particularly harmful.
In general, changes to thecell cycle, such as a non functionally
regulating system, can causethe cells to divide uncontrollably.
(03:05):
For example, mutated tumorsuppressor genes such as p53, a gene
responsible for apoptosis, maycause the cell to growth arrest and
cell death.
Oncogenes, which are mutatedproto oncogenes, can cause continuous
cell growth and division.
In the end, both the mutatedtumor suppressor gene and the oncogene
can cause cancer.
(03:26):
Although scientists are awareof these mutations, the exact causes
for the changes in the glialcells is unknown.
While scientists have notquite determined the exact cause
for dipg, more research iscurrently being conducted.
In a study by Michelle Mongyand other scientists, DIPG has been
linked to the dysregulation ofpostnatal development during middle
(03:47):
childhood and is connected toan active hedgehog signaling pathway.
The hedgehog signaling pathwayis important in vertebrate embryonic
development and it controlsthe formation and morphogenesis of
an organism.
Monge's experiment studiedcell populations with nestin, an
intermediate filament proteinin the ventral brainstem.
In the pons, during infancy,cells with NESTIN are present in
(04:10):
the ventral brainstem structure.
These cells decrease by theage of two.
However, in the human pons,the density of these cells peaks
around six years old, whichcorresponds to the incidence of dipg.
The hedgehog pathway is alsopart of the neural precursor cell
populations.
Scientists use betagalactosidase to determine the presence
(04:32):
of an active hedgehog pathway.
In the ventral ponds.
The beta galactosidasepositive cells were positive for
olig 2, a transcription factorknown to be regulated in the embryonic
spinal cord by the sonichedgehog gene and SOX2, a marker
of primitive cell types.
These cells increaseddramatically in middle childhood.
Thus, the hedgehog signalingpathway plays a role in the development
(04:54):
of dipg.
To ensure that the hedgehogsignaling pathway was causing the
cancer, the scientists usedthe sonic hedgehog protein ligand
which activates the hedgehogsignaling pathway and and they also
used a hedgehog antagonistwhich represses the pathway.
They then measured the redfluorescent protein produced from
the hedgehog pathway.
(05:14):
When the pathway was exposedto the hedgehog antagonist, the red
fluorescent protein decreasedby 67%.
When the sonic hedgehog ligandwas added, the red fluorescent protein
increased by 200%, suggestingthat the hedgehog pathway was indeed
involved.
Even though scientists found acorrelation between DIPG and an active
hedgehog pathway.
(05:35):
The dysregulation of thehedgehog pathway pathway doesn't
necessarily cause dipg.
This is evident since somediseases have inactive hedgehog pathways,
but the people do not develop dipg.
But a constantly activehedgehog signaling pathway is associated
with the proliferation of DIPG cells.
Now, the symptoms for DIPG arelargely dependent on where the tumor
(05:57):
forms, the size of the tumor,how fast the tumor grows, and the
child's age.
However, the symptoms developquickly and include trouble with
eye movements, visionproblems, problems with talking,
chewing and swallowing,drooping on one side of the face,
headaches, nausea andvomiting, weakness in the arms and
legs, loss of balance, troublewalking, changes in behavior and
trouble learning in school.
(06:19):
There are also many differentmethods for diagnosis.
A magnetic resonance imaging,also known as an mri, creates pictures
of areas inside the brain.
When gadolinium, a rare earthmetal used for MRIs, is injected
into a vein, it collectsaround cancer cells, making the cancer
more prominent in theOccasionally, a biopsy, specifically
(06:40):
a stereotactic biopsy thatuses imaging techniques to find and
remove the tumor tissue can be performed.
However, because of thelocation of the tumor, it isn't always
the best option.
Finally, immunohistochemistrycan also be done.
It uses antibodies to checkfor antigens in tissues and these
antigens are often used todetermine if the cancer is present.
(07:02):
Currently, there are no curesfor dipg, but there are treatments
that can be used to extend thepatient's lives.
Such treatments includeradiation therapy, chemotherapy and
surgery.
Radiation therapy uses highenergy X rays and other forms of
radiation to kill cancer cells.
Since this causes changes inbrain tissue, it can cause damage
to the brain, affecting growthand brain development.
(07:25):
Chemotherapy is another optionthat uses drugs to slow or stop the
growth of cancer cells.
It can either stop the cellsfrom dividing or kill them altogether.
Finally, surgery can be usedto place a shunt.
This is often needed forchildren with DIPG who have fluid
around their brain or spinal cord.
The shunt carries extra fluidaway from the brain, which ultimately
(07:46):
decreases the fluid and thepressure on the brain and spinal
cord.
Recently, scientists have alsofound several promising methods to
combat the cancer.
I'm going to talk about onespecific method that Ellum Smith
and other scientists have beenstudying, Synctron microbeam radiation
therapy.
Recently, scientists haveconsidered this microbeam radiation
(08:07):
therapy as a potentialalternative to the conventional radiation
therapy being used to treat dipg.
Microbeam radiation therapyhas been especially promising because
it spares normal brain tissuesby using spatial dose distribution.
This reduces the damage causedto the brain by conventional radiation
therapy and it slows down theprogression of the tumor.
(08:28):
Still, scientists have testedthe microbeam radiotherapy for efficiency
by examining apoptosis and theprogression of the cell cycle in
cancer cells that have beenexposed to it.
They found that in cell lineswith an active p53, the gene that
creates the proteinresponsible for apoptosis, apoptosis
of the cancer cells increases significantly.
(08:48):
Additionally, those cells werefound to have more cells in the sub
G0 phase, which is also knownas apoptosis, and decreased number
of cells in the G0, G1, G2 andM phases of the cell cycle.
Thus, apoptosis increased andthe cell cycle decreased after the
microbeam radiation.
(09:08):
For cell lines without afunctioning p53 gene, there was a
decrease in the cells in theG0 and G1 phase.
However, there was an increasein the sub G0 and G2 and M phases.
This accumulation of cells inthe G2 and M phases suggests that
there was a prolonged mitosis,so the DNA that was reduplicated
(09:29):
was not separated correctlyand accumulated in the wrong phase.
Since the DNA was notseparated correctly, polyploidy,
which occurs when an organismhas the wrong number of chromosomes,
occurred.
Ultimately, these polyploidcells were reproductively dead, resulting
in very little cell duplication.
Thus, because of theseresults, microbeam radiotherapy proves
(09:49):
to be an alternativeradiotherapy treatment for patients
with dipg because it is moredamaging for cancer cells and less
damaging for normal tissues.
While DIPG is still underresearched, the current research
being conducted by scientistshave led to major breakthroughs about
the causes and potential curesfor the cancer.
There is still a lot of workthat must be done, but a future cure
(10:10):
for DIPG is currently in the works.
Thank you for listening.
Popular Podcasts
NFL Daily with Gregg Rosenthal
Gregg Rosenthal and a rotating crew of elite NFL Media co-hosts, including Patrick Claybon, Colleen Wolfe, Steve Wyche, Nick Shook and Jourdan Rodrigue of The Athletic get you caught up daily on all the NFL news and analysis you need to be smarter and funnier than your friends.
On Purpose with Jay Shetty
I’m Jay Shetty host of On Purpose the worlds #1 Mental Health podcast and I’m so grateful you found us. I started this podcast 5 years ago to invite you into conversations and workshops that are designed to help make you happier, healthier and more healed. I believe that when you (yes you) feel seen, heard and understood you’re able to deal with relationship struggles, work challenges and life’s ups and downs with more ease and grace. I interview experts, celebrities, thought leaders and athletes so that we can grow our mindset, build better habits and uncover a side of them we’ve never seen before. New episodes every Monday and Friday. Your support means the world to me and I don’t take it for granted — click the follow button and leave a review to help us spread the love with On Purpose. I can’t wait for you to listen to your first or 500th episode!
Dateline NBC
Current and classic episodes, featuring compelling true-crime mysteries, powerful documentaries and in-depth investigations. Follow now to get the latest episodes of Dateline NBC completely free, or subscribe to Dateline Premium for ad-free listening and exclusive bonus content: DatelinePremium.com