DIPG or diffuse intrinsic pontine glioma is a type of tumor that starts in the brain stem, the part of the brain just above the back of the neck and connected to the spine. The brain stem controls breathing, heart rate and the nerves and muscles that help us see, hear, walk, talk and eat. These tumors are called gliomas because they grow from glial cells, a type of supportive cell in the brain.
What are the stages of DIPG?
DIPG falls into the Glioma staging system, so they can be classified according to the four stages below based on how the cells look under the microscope. The grades are from the least severe to the most severe.
- Low Grade: Grade I or II means that the tumor cells are the closest to normal.
- High Grade: Grade III or IV means that these are the most aggressive tumors.
The main issue with DIPG is that most of these tumors are not classified by grade because surgery to obtain tissue by biopsy or to remove the tumor is not safe because of the location of the tumor. When these tumors are biopsied, they are usually grade III or grade IV tumors, which tend to behave very aggressively. Most of the tumors are diagnosed by their appearance on MRI.
How common is DIPG?
- About 10% to 20% of all childhood brain tumors are DIPG or brainstem gliomas.
- They are more common in children between the ages of 5 and 10 years, but can occur at any age in childhood. Though more rare, they can also occur in adults.
What are the symptoms of DIPG?
Symptoms usually develop rapidly in the majority of patients because of the fast growth of these tumors.
The most common symptoms related to diffuse intrinsic pontine glioma include the following:
- Problems with balance and walking
- Problems with the eyes (including double vision, drooping eyelids, uncontrolled eye movements, blurred vision)
- Problems with chewing and swallowing
- Nausea and vomiting
- Morning headache or headache that gets better after the child vomits
- Facial weakness or drooping (usually one side)
How is DIPG treated?
- Radiation therapy — currently, is the primary therapy for newly diagnosed DIPG in children older than 3. It uses high-energy X-rays from a specialized machine or other types of radiation to kill cancer cells and shrink tumors (stop them from growing). Radiation provides a temporary response in most patients but does not provide a cure.
- Chemotherapy — is being used along with radiation therapy and other biologic agents in multiple trials to decide if we can find ways to improve the survival rates of patients with DIPG.
- Surgery — is rarely used to diagnose DIPG because of the risks involved in surgery in this area of the brain. There are cases where a biopsy can be obtained safely, but the use of surgery to remove the tumor is very rare.
- A biopsy (removal of affected tissue to be examined) may be done to decide the tumor’s type and grade.
- If the biopsy finds cancer cells and the patient is still in surgery, the surgeon may take out as much tumor as can be removed safely. Depending on where the tumor is located, this might not be an option.
What are the survival rates for DIPG?
Unfortunately, the survival rate for DIPG remains very low. At this time there is no silver bullet for this disease. There are clinical trials that can help prolong the life of these children. Please contact us if your interested in assistance in locating a clinical trial in your area.
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