Diffuse Intrinsic Pontine Glioma (DIPG)

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Diagnosing and Treating DIPG

The brainstem consists of three parts: The midbrain, the pons, and the medulla oblongata. DIPG is a pontine glioma, meaning that it develops in the pons.

Unlike tumors at other locations, a tumor in the pontine segment of the brainstem is not often biopsied. Diffuse intrinsic pontine glioma (DIPG) is usually diagnosed based on the patient’s symptoms and magnetic resonance imaging (MRI) studies. MRI scans produce detailed images of the brain and allow doctors to detect the presence of a tumor. Sometimes the patient is also given a contrast agent (a special dye) before the scan to help doctors better tell whether the abnormal tissue is a tumor and what type of tumor it is. The location in the pons, the way it infiltrates nearby tissue, and the lack of clear borders of the tumor all signify that a tumor is likely DIPG.

If the child’s symptoms and the MRI images are not typical, a stereotactic biopsy may be required to make a diagnosis. In this case, a small hole is drilled in the skull and a needle is passed through the hole to take a small piece of tissue for testing. A pathologist will examine the sample and make a diagnosis.

Treatment for DIPG
Although surgical resection is the treatment of choice for many other types of brain tumors, it is not considered a treatment option for children with DIPG. The tumor infiltrates so extensively into normal brain tissue, and the brainstem carries out such critical functions (like controlling breathing), that surgical removal of the tumor is impossible. Chemotherapy has not been proven effective, at least in part because anti-cancer drugs cannot cross the blood-brain barrier and reach the tumor in sufficient amounts.

Radiation therapy is the primary treatment for DIPG. Radiation therapy uses high-energy X-rays directed at the tumor to help shrink it and reduce pressure on the brain. This is typically performed as five daily sessions per week for a total of 30 to 33 sessions. This treatment can alleviate symptoms quickly and hold the cancer at bay for about six months on average. When the tumor comes back, repeat radiation therapy is an option. Repeat radiation therapy is usually done with five daily sessions (but the dose per session is different from that of initial radiation therapy) per week for 10 sessions. Repeat radiation therapy on average gives the patient four more months of time.

There are a number of clinical trials for DIPG. One potential new treatment now in clinical trial at the Weill Cornell Medicine Brain and Spine Center and Memorial Sloan-Kettering involves the delivery of an anti-cancer agent directly to the tumor. Injecting this targeted radio-immunotherapeutic drug directly into the tumor avoids intruding on delicate brain stem tissue and eliminates the need for medicine to cross the blood-brain barrier. Dr. Souweidane recently published the long-awaited results of this Phase 1 trial, and you can see his results here

Find out more about the clinical trial.

Find out more about the Children’s Brain Tumor Project.

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Reviewed by: Zhiping Zhou, Ph.D.
Last reviewed/last updated: June 2018
Illustration by Thom Graves, CMI