Children with diffuse intrinsic pontine glioma (DIPG) are treated through the Glioma Program at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, an integrated pediatric hematology and oncology partnership between Dana-Farber Cancer Institute and Boston Children’s Hospital. Working together, we provide more specialists, more programs, and more clinical trials than almost any other pediatric center treating cancer and blood disorders in the world.
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 your child’s 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 intrinsic pontine glioma?
Treatment for DIPG may include:
- radiation therapy – This is the primary therapy for newly diagnosed DIPGs. 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 more than 90 percent of children with DIPGs. These responses are short-lived, however, lasting about six to nine months on average. 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 for this condition. Several trials evaluating new agents are either underway or have been recently completed. With a biopsy of the tumor at diagnosis, the selection of drugs targeted to your child’s tumor can be determined.
In addition, there are trials evaluating whether new ways of delivering the traditional drugs might improve responses. Multiple clinical trials have demonstrated that routine chemotherapy does not increase survival rates in this condition.
Unfortunately, complete surgical removal is not an option in the treatment of these tumors, because they occur in the brainstem. Surgery in this part of the brain can cause severe neurological damage and affect the body’s most vital functions. When needed, it must be performed by an experienced pediatric neurosurgical team such as the one in the Dana-Farber/Boston Children’s team.
How are side effects of pediatric cancer treatment managed?
Side effects in the treatment of diffuse intrinsic pontine glioma can arise from biopsy, radiation and chemotherapy.
- Procedures should be performed in specialized centers where experienced neurosurgeons, working in the most technologically advanced settings, can provide the most precise molecular diagnosis while preserving normal brain tissue.
- Radiation therapy often produces inflammation, which can temporarily worsen symptoms and dysfunction. To control this inflammation, steroids are sometimes necessary. New agents now are being tested that control the tumor- and radiation-associated swelling without causing the typical side effects of steroids.
- 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 the side effects of therapy, include the following.
Talk to your child’s physician about whether complementary or alternative medicine might be a viable option.
What is the expected outcome after treatment for diffuse intrinsic pontine glioma?
Unfortunately, the prognosis for DIPGs remains very poor, although a small percentage of patients 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 DIPG. Current trials include new medications as well as new methods for the delivery of more traditional agents.
Resources and support
There are also a number of resources and support services at Dana-Farber/Boston Children's Cancer and Blood Disorders Center to help you and your family through this difficult time.
When appropriate, our Pediatric Advanced Care Team (PACT) offers supportive treatments intended to optimize the quality of life and promote healing and comfort for children with life-threatening illness. PACT also can provide psychosocial support and help arrange end-of-life care, if necessary.