Treatment of DIPG

DIPGs are relatively rare, and the diagnosis and treatment of DIPG is complex and involves multiple specialists. Patients and families should look to comprehensive, experienced pediatric medical centers with dedicated pediatric neuro-oncologists, neurologists, neuroradiologists, and neurosurgeons to receive a definitive diagnosis and care.


Surgery

DIPG grows in a way that makes surgical removal impossible. The cancer tissue spreads into healthy tissue, and the edges of the tumor are not well defined. Surgeons cannot remove the tumor because there is no way to avoid cutting out healthy brain tissue which is necessary for survival.


Radiation

Radiation therapy is the only proven beneficial treatment but unfortunately for limited duration. The radiation kills some of the cancer cells, shrinking the tumor and reducing pressure on the brainstem. This can greatly improve how the child feels and functions for a few months, and 75-85% of patients show improvement in symptoms after radiation therapy. Because even carefully aimed radiation therapy causes some damage to the healthy brain tissue surrounding the tumor, there is a limit to how much radiation therapy can be given to each patient. Unfortunately, the cancer almost always grows back after a few months.

Proton Beam Therapy

Proton beam therapy is a specific type of radiation therapy that uses proton particles for treatment rather than the photons (x-rays) that are used for most radiotherapy. Protons have an anti-tumor effect that is very similar to conventional photon radiotherapy. In some brain tumors, protons are superior to photon radiation because they can spare nearby normal brain tissue from potentially harmful effects of radiation. However, they have not been shown to have an advantage in the treatment of DIPG.

Radiation Side Effects

Radiation therapy often causes inflammation in the brain, which can temporarily make symptoms worse. Steroids are used to control inflammation when necessary.


Chemotherapy

Current chemotherapy is generally not effective against DIPG. Most cancer-killing drugs are not able to get into the tumor at all, which seems to be protected by the high pressure inside the pons and by the blood-brain barrier, which prevents most drugs given orally or intravenously from reaching the brain.

A series of clinical trials have tested various chemotherapy drugs and combinations of drugs before or along with radiation therapy, but so far, no trial has shown that chemotherapy extends life. Trials of newer molecularly targeted agents and anti-angiogenic agents (drugs that should prevent tumors from forming new blood vessels to feed their growth) have also so far failed to show any significant benefit to patients.

Researchers are studying drugs that may be able to pass through the blood-brain barrier and are also investigating ways to inject chemotherapy drugs directly into the tumor. Work to discover new drugs also continues. As researchers learn more about the causes and biological mechanisms of DIPG, they will be able to design more effective chemotherapies to fight these tumors.

Chemotherapy Side Effects

Some chemotherapy drugs can cause side effects such as diarrhea, constipation, fatigue, and headache. Usually these side effects can be controlled with additional medications.