Diffuse pontine glioma

We know how difficult a diagnosis of a pediatric brain tumor can be, both for your child and for your whole family. That’s why our physicians are focused on family-centered care: From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs. We’ll work with you to create a care plan that’s best for your child. 

If your child has been diagnosed with a diffuse pontine glioma, you’ll naturally be eager to know how your child’s physician will treat the tumor. Your child’s physician will determine a specific course of treatment based on several factors, including:

• your child's age, overall health and medical history
• type, location, and size of the tumor
• extent of the disease
• your child's tolerance for specific medications, procedures or therapies
• how her doctors expects the disease to progress

There are a number of treatments we may recommend. Some of them help to treat the tumor while others are intended to address complications of the disease or side effects of the treatment.

What are the treatments for a diffuse pontine glioma?

If your child has been diagnosed with a diffuse pontine glioma, treatment may include:

• radiation therapy – This is the primary therapy for newly diagnosed diffuse pontine glioma. It uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors. Conventional limited-field radiation produces responses in over 90 percent of children with diffuse pontine gliomas. These responses are short-lived however, with a median duration of about 6 months. Several trials to increase the dose of radiation therapy have been performed and none have improved survival.
   
• experimental chemotherapy – Chemotherapy and biologic therapy in combination with radiation therapy is actively being investigated as a treatment of this condition. Several trials evaluating new agents are either underway or have been recently completed.

In addition, there are trials evaluating whether new ways of delivering the traditional drugs might improve responses. We should know more about the results of these tests soon. Unfortunately, no currently available chemotherapy regimen has been shown to increase survival rates in this condition.

Unfortunately, complete surgical resection is not an option in the treatment of these tumors because of where the tumor is located. Diffuse pontine gliomas occur in the brainstem, which controls the body’s most vital functions. Surgery in this part of the brain can cause severe neurological damage.

How are side effects managed?

Side effects in the treatment of diffuse pontine glioma can arise from radiation and chemotherapy.

• Procedures should be performed in specialized centers where experienced neurosurgeons, working in the most technologically advanced settings, can provide the most extensive resections while preserving normal brain tissue.
   
• Radiation therapy often produces inflammation, which can temporarily exacerbate symptoms and dysfunction. To control this inflammation, steroids are sometimes necessary.
   
• Some of the chemotherapy agents are associated with fatigue, diarrhea, constipation and headache. These side effects can be effectively managed under most circumstances.

Many specialized brain tumor treatment centers now have specialists who deliver complementary or alternative medicines. These treatments, which may help control pain and side effects of therapy include the following.

• acupuncture/acupressure
• therapeutic touch
• massage
• herbs
• dietary recommendations

Talk to your child’s physician about whether complementary or alternative medicine might be a viable option.

In the event that end-of-life care is necessary, our Pediatric Advanced Care Team (PACT) is available to ease symptoms and help your child maintain quality-of-life as much as possible.

What is the expected outcome after treatment for diffuse pontine glioma?

Unfortunately, the prognosis for diffuse pontine gliomas remains very poor, although a small percentage of patients can survive this disease. Your child’s physician will discuss treatment options with you, including experimental clinical trials and supportive care.

What about progressive or recurrent disease?

Clinical trials and experimental therapies are available for patients with relapsed diffuse pontine glioma. Current trials include novel medications as well as new methods for the delivery of more traditional agents.

Resources and support

We understand that you may have a lot of questions if your child is diagnosed with a diffuse pontine glioma. We’ve tried to provide some answers to those questions in these pages, but there are also a number of resources and support services to help you and your family through this difficult time.