Diffuse Intrinsic Pontine Glioma (DIPG) is a tumor located in the pons (middle) of the brain stem. The brain stem is the bottom most portion of the brain, connecting the cerebrum with the spinal cord. The majority of brain stem tumors occur in the pons (middle brain stem), are diffusely infiltrating (they grow amidst the nerves), and therefore are not able to be surgically removed. Glioma is a general name for any tumor that arises from the supportive tissue called glia, which help keep the neurons ("thinking cells") in place and functioning well. The brain stem contains all of the "wires" converging from the brain to the spinal cord as well as important structures involved in eye movements, face and throat muscle control and sensation.
Diffuse Intrinsic Pontine Gliomas are located in the brainstem, at the base of the brain. They are usually diagnosed in children aged 5 to 10. They are difficult to treat because the tumor cells grow in between and around normal cells. It is impossible to remove a tumor in this area because it interferes with the functioning of this critical area of the brain.
What causes a Diffuse Intrinsic Pontine Glioma?
We don’t know what causes a Diffuse Intrinsic Pontine Glioma. There is no way to predict that a child will get brain cancer and nobody is to blame if a child develops a tumor. Researchers have been studying whether environmental factors, such as radiation, food, or chemicals can cause brain cancer. At the moment, there is no definite proof that there is a connection.
How many other children have Diffuse Intrinsic Pontine Gliomas?
Diffuse Intrinsic Pontine Gliomas account for about 5-10 out of every 100 brain tumors in children.
What are the medical symptoms of a Diffuse Intrinsic Pontine Glioma?
Some symptoms are muscle weakness on one side of the body, swallowing problems, speech problems, crossed eyes, drowsiness, hearing loss, and personality changes. The brainstem has cranial nerves that control many of these functions. The tumors affect these nerves and cause symptoms.
What is the outcome for a child with Diffuse Intrinsic Pontine Glioma?
Because they are difficult to treat, the outcome for brainstem gliomas is poor. After diagnosis, the survival time is on average 9 to 12 months. To improve the outcome, doctors have tried giving higher amounts of radiation, or using chemotherapy medicines to kill the tumor cells. Research is underway to achieve better results. When the tumor recurs, the focus of treatment is on managing symptoms to make sure the child is as comfortable as possible.
How is a diffuse intrinsic pontine glioma treated?
These tumors are treated with radiation therapy, which are high-energy X-rays that destroy tumor cells. This treatment can reduce symptoms significantly, but there may be some permanent damage caused by the tumor which can’t be helped. Steroids, another type of drug, are often given to improve some of the symptoms. Surgery is not part of the standard treatment because the tumor has grown within a part of the brain where resection is impossible. The effectiveness of chemotherapy is still uncertain.