Diffuse intrinsic pontine glioma (DIPG) is an aggressive type of childhood cancerous tumor that arises from the pons or brain stem (the part of the hindbrain). The brain stem controls important functions such as breathing, heart rate, and muscle tone. The exact cause of DIPG is unknown. It is a rare fast-growing cancer. Gliomas arise from glial cells, the cells found throughout the nervous system. They surround and support the nerve cells. DIPG is considered fatal cancer. The 2-year survival rate typically is 10%, and the 5-year survival rate is around 2%.
However, a small number of patients with DIPG tumors may have better outcomes than others. These are the tumors bearing mutations in a gene that is involved in the basic packaging of DNA. These mutations are known as histone mutations.
Grading of DIPG: DIPG is graded based on the nature of the tumor. The chances of survival depend on the grade.
- Grades I and II tumor cells are the closest to normal cells, and they are less aggressive.
- Grades III and IV are the most aggressive tumors. A grade III tumor is anaplastic, and a grade IV glioma is also called glioblastoma.
What are the signs and symptoms of DIPG cancer?
Common signs and symptoms of diffuse intrinsic pontine glioma (DIPG) differ as per the area of the brain stem from where the cancer arises. The following are the commonest symptoms of DIPG:
- Difficulty maintaining balance, standing, and walking
- Double vision
- Drooping eyelids
- Uncontrolled eye movements
- Blurred vision
- Difficulty chewing
- Difficulty swallowing
- Nausea and vomiting
- Headache or headache that improves after the child vomits
- Facial weakness
- Deviation of angle of the mouth to one side
- Dribbling of saliva
- Unusual sleepiness
How is DIPG cancer treated?
Because diffuse intrinsic pontine glioma (DIPG) and tumors that bear this type of mutation are so rare and it is difficult to perform their biopsy, clinical guidelines are based on relatively small patient studies. Treatment options include:
- Radiation therapy: This is the main treatment for children aged older than 3 years with newly diagnosed DIPG. The treatment involves using high-energy X-rays that kill cancer cells and shrink tumors. Radiation therapy is usually not curative.
- Chemotherapy: This involves using powerful medication that may be taken orally or injected to destroy cancer cells. It may be used along with radiation therapy, but this may also not be curative.
- Surgery: Surgery is rarely performed to remove DIPG. The location of the tumor carries a high risk of fatal complications.
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