DIPG Treatment Overview

Once the diagnosis of DIPG is suspected, there is a standard course of treatment that your doctor will most likely follow:

  • At the time of diagnosis, steroids are often the first treatment offered to patients with diffuse intrinsic pontine glioma.
  • Soon after the steroid regimen, patients are prepped for radiation therapy.
  • Unfortunately, no chemotherapy drugs to date are known to have an impact on survival.

In almost all cases, after about 6 to 12 months, the DIPG tumor starts to grow again. Once the tumor has started to grow again, no further treatment has been shown to improve survival. When children start to have neurologic symptoms, they are often restarted on steroids.

More on the Clinical Course of DIPG

Steroids

Steroids are often used in the management diffuse intrinsic pontine gliomas (DIPGs).  Steroid treatment aims to improve neurological symptoms, reduce the edema surrounding the tumor that may sometimes impact the flow of the cerebrospinal fluid (CSF), and prevent or minimize the edema induced by the initiation of the radiation treatment.  Side effects from steroids can include hyperphagia—a feeling of extreme excessive hunger, immunosuppression, anxiety, insomnia, irritability, steroid myopathy, gastrointestinal issues, and fluid retention.

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Radiation

Radiation therapy is the standard treatment for children with DIPGs.  It is an effective palliative treatment that improves symptoms in about 80% of children with DIPGs, prolonging their survival by 2-3 months.  A common complication of radiation therapy in children with a DIPG is radiation necrosis—cell death of brain tissue.  Steroids are typically used for the symptomatic treatment of radiation necrosis.

Medical professionals are exploring the use of radiosensitizers, which are agents that may be given along with radiation to improve the effects of this therapy.

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Surgery

Operating on DIPGs is an issue because they are diffuse, intrinsic and in the pons.  DIPG cells don’t grow as large masses that push normal brain tissue aside, instead they are lumps of cells that grow into surrounding tissue, therefore mixing cancer cells with healthy cells. As a result, it’s not possible for a surgeon to separate normal from abnormal tissue.  In some cases, children with DIPGs will need to have procedures to control cerebrospinal fluid pressure in the brain caused by DIPG growth.

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Chemotherapy

Chemotherapy is any medication that kills cancer cells.  It works by targeting cells that are actively dividing thereby stopping the cancer cells from reproducing.  There are a couple major challenges that exist with brain tumors.  One is the blood-brain barrier, which blocks many unrecognizable, potentially toxic substances including many types of chemotherapy from penetrating the brain.  Another challenge is that the tumor cells that make up brainstem gliomas are resistant to chemotherapy.

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CED (Convection Enhanced Delivery)

Convection Enhanced Delivery (CED) is a technique of inserting a small catheter directly into the DIPG, the pontine tumor, to deliver drugs.  CED is a way to bypass the Blood Brain Barrier and deliver drugs at high concentrations into the DIPG tumor.

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Clinical Trials

Clinical trials are used to test new possible treatments for cancer and to find out whether a treatment is safe; whether a treatment has the effects on cancer cells that scientists think it will; and whether it is effective at shrinking tumors, delaying growth of tumors, or making people live longer.  Clinical trials are a vital part of medical research, and without them new treatments that may save or improve the lives of cancer patients would not be identified.

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Emerging Treatments

New and upcoming treatment methods for DIPG include, Proton Beam Radiation, Intra-Arterial Chemotherapy, and Immunotherapy.  With more clinical trials and studies on these emerging methods, DIPG treatment options may expand.

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