Diffuse Infiltrative Pontine Glioma (DIPG)
Diffuse Infiltrative Pontine Glioma, or DIPG, is a rare type of inoperable, incurable brainstem tumor. The name refers to the nature and location of the tumor:
- It is diffuse, meaning it's not well defined. Tumors with clear definition are better candidates for surgical removal than diffuse ones.
- Infiltrative means that it invades nearby brain cells, causing neurological symptoms and making it even more difficult to treat.
- "Pontine" means that it occurs in an area of the brainstem (the "pons") that controls many critical functions, including breathing.
- It's a glioma, meaning it arises from glial (supporting) cells.
These four factors combine to make these tumors impossible to remove surgically. Chemotherapy is largely ineffective, since anti-cancer drugs cannot cross the blood-brain barrier and reach the tumor. Radiation is the most common treatment, but its benefit is temporary, measured in months. Unfortunately, DIPG is currently considered incurable.
There are about 200 new cases of DIPG diagnosed each year, usually in children under age 10.
Symptoms
The most common initial symptoms associated with DIPG (as well as other brainstem gliomas) are:
- Difficulty walking
- Headache
- Nausea
- Vomiting
- Double vision
- Trouble swallowing
- Facial weakness
Because the brainstem is adjacent to the brain structures that control blood pressure, breathing, and a number of motor and sensory functions, pontine gliomas can cause significant neurological problems. The location of these tumors also means that many children with them may experience hydrocephalus, a condition in which the flow of cerebrospinal fluid around the brain is blocked, causing an increase in pressure inside the skull that results in headache, nausea, and vomiting. Also, these tumors may grow large before symptoms begin.
Diagnosis
A pontine glioma is diagnosed by imaging studies. Magnetic resonance imaging (MRI) and computed tomography (CT) scans can identify the tumor and determine its relationship to adjacent structures. A contrast agent is administered intravenously so neurological surgeons can visualize the tumor against the normal brain in the background.
Treatment
Although surgery is the treatment of choice for many other types of brain tumors, it's usually not possible for children with DIPG because this region can be difficult to reach surgically, the tumors are close to critical areas of the brain, and they infiltrate normal tissue. Initial treatment may include a surgical procedure to reduce pressure inside the skull caused by hydrocephalus. Most often the pressure is reduced with a shunt, an implantable tube that allows the excess fluid to drain to other parts of the brain or elsewhere in the body. Also, drugs such as corticosteroids may be given to help reduce symptoms.
Radiation therapy is the primary treatment for DIPG, but it may have adverse effects on development, and its benefits are short-term. Chemotherapy is not widely used in children with DIPG because its efficacy has not been established, and because
One promising new treatment now in clinical trial involves the delivery of an anti-cancer agent directly to the tumor. Injecting this targeted radio-immunotherapeutic drug directly into the glioma avoids intruding on delicate brain stem tissue, and eliminates the need for medicine to cross the blood-brain barrier. Find out more about this clinical trial.
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