Diffuse Intrinsic Pontine Glioma (DIPG)
Diffuse Intrinsic Pontine Glioma (DIPG) is a type of tumor that can grow in the brain or spinal cord.
Diffuse Intrinsic Pontine Glioma
Watch this short video about DIPG treatment at Nationwide Children's Hospital
What Is Diffuse Intrinsic Pontine Glioma (DIPG)?
Diffuse intrinsic pontine glioma, also known as DIPG, is an aggressive, malignant (cancerous) brain tumor that develops in a part of the brainstem called the pons. The pons is the portion of the brain that helps send messages to the spinal cord and the rest of the body, controlling important functions like heartbeat, breathing, sleeping, balance and bladder control. As these tumors grow, they put pressure on other parts of the brain and can impact function of nerves that control muscles and sensation of the face.
Around 300 children are diagnosed with DIPG each year, and it is most common in children ages 5-9. There is currently no cure for DIPG, but researchers at Nationwide Children’s and elsewhere are working hard to explore new ways to treat this disease.
What Causes DIPG?
There is no known cause for DIPG. There is no evidence that any environmental factors cause this type of tumor, and it is not a condition that is passed down to offspring. Researchers believe that something is absent during normal development and growth. Specifically, cells of the brain and spinal cord grow and divide as the body develops. During this cell division process, cells need to replicate their genetic material. Errors can take place during this process, leading to mutations (changes in the gene structure), which may allow ependymal cells to grow into tumors. These errors generally occur randomly and cannot be prevented.
What Are the Signs and Symptoms of DIPG?
Signs and symptoms in your child may appear and progress suddenly due to rapid growth of the tumor. Some of the symptoms you may see in your child include:
How Is DIPG Diagnosed?
Typically, DIPG tumors are diagnosed by magnetic resonance imaging (MRI) and based upon your child’s symptoms. In some cases, your child’s doctor may recommend a biopsy to learn more about the tumor, but this is not always performed.
- MRI - An MRI uses large magnets, radio waves and a computer to make detailed pictures of the body. Contrast dye may be injected into your child's vein. MRIs are detailed imaging (provide a lot of information about the tumor), so take about 1-2 hours to perform.
- Biopsy - Tumor cells are removed during surgery and sent to a lab for testing, to learn more about the tumor (how it appears under the microscope) and for possible tumor genetic testing. The surgeon may use an imaging tool to help them guide the biopsy needle to the right location.
How Are DIPG Tumors Treated?
There is sadly no proven curative treatment for patients with DIPG, though this remains a huge area of ongoing research. Due to the location of the tumor, complete removal by surgery is not a viable, safe option. The tumor is in the brainstem, which helps control basic life functions. It is not possible to fully remove the tumor without damaging these healthy and important brain cells.
Most patients are treated with radiation therapy (high-energy x-rays which are used to kill or slow down tumor cells); although radiation sadly cannot cure DIPG, it has been shown to increase survival and improve symptoms temporarily in most patients.
Chemotherapy alone has not been shown to be effective in treating patients with DIPG, but there are several ongoing clinical trials exploring many different types of medical therapy alone or in combination. Talk with your child’s doctor about whether any open clinical trials may be a good fit for your child (see below).
The average survival of patients with DIPG is tragically less than one year from diagnosis; however, this varies from patient to patient depending on several factors that are still being understood, and with hope of future improvement with ongoing research.
Why Choose Nationwide Children's?
The Neuro-Oncology Program at Nationwide Children’s Hospital offers clinical excellence in treating children, adolescents, and young adults with brain and spine tumors. Your child will have access to doctors who are experts in different types of medical care. We call this multidisciplinary care. Depending on the specific tumor diagnosis and treatment plan, your team may include oncologists, neurosurgeons, radiation oncologists, clinical psychologists, rehabilitation medicine doctors, neurologists, endocrinologists, ophthalmologists, therapists (physical, occupational, speech, art, music), social workers, dietitians and/or pharmacists. These team members all work together with the family to provide the best care for the patient.
Cancer Clinical Research
Nationwide Children’s is a national leader in oncology research and clinical trials. There are many ongoing research studies aimed at improving outcomes for children and young adults with brain/spine tumors—understanding why these tumors develop and how to appropriately target them. We are members of all major pediatric brain tumor clinical trial consortia including Pediatric Brain Tumor Consortium (PBTC), Collaborative Network of Neuro-oncology Clinical Trials (CONNECT), Pacific Neuro-oncology Consortium (PNOC) and the Children’s Oncology Group (COG). We have many open clinical trials testing new drugs in difficult-to-treat tumors, including in DIPG.
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