Diffuse intrinsic pontine glioma, or DIPG, accounts for about 10 percent of all childhood central nervous system tumors and 80 percent of children’s brainstem tumors. These fast-growing tumors are usually diagnosed in children who are between the ages of 5 and 9, but they can affect children of any age. Because of the particular nature and location of these tumors, diagnosing and treating them poses special challenges.
Gliomas, or glial tumors, arise in the brain’s glial tissue, which is made up of cells that help protect the brain’s neurons, which are the specialized cells responsible for transmitting information. Diffuse intrinsic pontine gliomas occur in the pons, an area of the brainstem that controls the nerves relating to eye movement, sensation in the face, hearing, chewing and swallowing. This part of the brainstem is also responsible for several major functions needed for life, such as heartbeat, blood pressure and breathing.
Many kinds of tumors occur as a solid mass, but DIPGs are very different in form. These tumors spread around healthy brain tissue and displace that healthy tissue as they grow. Until recently, researchers believed that DIPGs in children and adults were similar, but new studies suggest that the tumors that occur in children’s brains may be their very own entity. These findings may eventually lead to new and more individualized treatment options for children diagnosed with DIPG.
Common Symptoms of DIPG
The symptoms of DIPG can develop very rapidly. Because this type of tumor affects the pons, with its many essential functions and nerves, these symptoms typically include:
- Problems with eye movements and vision
- Difficulty with facial expressions and movements
- Sudden problems with speech, chewing and swallowing
- Weakness in the arms or legs
- Problems with walking and coordination
- Headaches, often accompanied by vomiting
Making a diagnosis of DIPG begins with a medical history and physical examination. Other tests can include computerized tomography (CT), magnetic resonance imaging (MRI) or magnetic resonance spectroscopy (MRS), both of which can help to identify the type of tumor.
One common approach to diagnosing many tumors is biopsy, a procedure that involves collecting tissue or cells from the tumor for examination. Because of the location of diffuse intrinsic pontine gliomas, performing a biopsy can be risky and difficult. However, recent advances in imaging and technology now make it possible for neurosurgeons to biopsy DIPGs in some cases, and this can help with staging the tumor and developing a treatment plan. In most cases, though, DIPGs are typically diagnosed by MRI or other noninvasive imaging procedures.
Treatments for DIPG
Standard treatments for cancers in the brain include surgery, radiation and chemotherapy. But in the case of DIPGs, surgery is generally not an option, since these tumors are interspersed with healthy brain tissue. Although biopsies may be done safely, surgery in the delicate area of the brainstem can cause severe neurological damage or affect the processes needed for life.
For these reasons, radiation therapy is the preferred treatment for DIPGs in children older than 3 years. Conventional photon beam radiotherapy is delivered in an outpatient setting. In this type of treatment, radiation is directed at the brainstem and surrounding tissues. An alternative form of radiation therapy, known as proton beam radiotherapy, may be preferable in some cases, since this particular kind of radiation spares more of the normal tissue surrounding the tumor.
Radiation therapy is painless and noninvasive, but it can cause inflammation in the brain. This inflammation can temporarily make symptoms worse, so patients may be given a course of steroid medications to control it. Radiation can also cause longer-term neurological problems because of its effect on healthy brain tissues.
Radiation therapy has been shown to lead to an improvement in symptoms in up to 90 percent of patients, and these improvements can often last for several months. But in most cases, the tumor eventually returns, affecting virtually all neurological functions and depressing essential processes such as heart rate and breathing.
Chemotherapy can be used in conjunction with radiation to shrink tumor cells. Chemotherapy may be given orally or intravenously and is usually provided in several cycles on an outpatient basis. The side effects of chemotherapy are temporary; they can include hair loss, nausea or vomiting.
Diffuse intensive pontine glioma is an aggressive brainstem tumor and a challenging one to treat. Ongoing research continues to reveal new insights into the unique nature of these tumors, offering the possibility of new therapies and treatments to help reduce symptoms and improve outcomes for children with this condition.
Why Choose Neurosurgeons of New Jersey?
With the largest subspecialized neurological practice in the tri-state area, Neurosurgeons of New Jersey is a leader in both cutting-edge technology and patient-centered care. The center’s specialists in pediatric neurosurgery and other disciplines bring decades of experience and state-of-the-art treatment options to address each patient’s individual circumstances. At Neurosurgeons of New Jersey, your neurosurgeons and health care team work will with you to develop the treatment plan that’s right for your child.