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What is a DIPG?

A Diffuse Intrinsic Pontine Glioma (DIPG) is a malignant tumor located in the pons or middle portion of the brain stem. The brain stem, located at the bottom of the brain, connects the brain to the spinal cord. These tumors are diffusely infiltrating, growing amidst the nerves, rendering surgical removal impossible. Glioma is general term describing a tumor arising from the glial or supportive tissue that helps keep the neurons or “thinking cells” in place and functioning. The brain stem contains all the “wires” converging from the brain to the spinal cord as well as important structures involved in eye movement and coordination, face and throat muscle control, and sensation.

Approximately 150 to 200 cases of DIPG are diagnosed annually. The median age at diagnosis is 5 to 9 years old but tumors can develop at any age in childhood. DIPGs occur with equal frequency among boys and girls, and there are no known factors or conditions that predispose a child to developing DIPG.

Symptoms

DIPG causes symptoms related to the nerves that support the muscles of the eyes and face, and muscles involved in swallowing and breathing. Although each child may experience them differently, symptoms commonly include double vision, an inability to completely close the eyelids, facial drooping on one side, and difficulty chewing or swallowing. DIPGs also affect the “long tracks” of the brain resulting in weakness of the arms or legs and difficulty with speech and walking. Other related symptoms include headache, fatigue, and vomiting. Symptoms typically advance rapidly due to the aggressive growth of the tumor, with most children experiencing symptoms less than three months and many less than three weeks prior to diagnosis.

Diagnosis

Diagnosis is based upon symptoms and a radiological study such as a CT scan or more commonly MRI. Clearly defined features of DIPG tumors make MRI diagnosis highly accurate.

Treatment

Treatment of DIPG has advanced very little over the last thirty years. Standard treatment includes steroids and six weeks of radiation therapy. Radiation treatment utilizes high-energy rays from a specialized machine to damage or kill cancer cells and shrink tumors. Conventional limited-field radiation produces responses in over 90% of cases. Although most children experience a profound improvement during and after radiation treatment, symptoms usually recur in six to nine months and progress quickly.

Clinical trials of chemotherapy and biologic therapy in combination with radiation are being actively investigated by researchers. Unfortunately, no currently available drug therapy has been shown to increase survival rates.

Survival Rates

The prognosis for children with DIPG is grim. The median survival time is less than 1 year, and the 2-year survival rate is less than 20%. Longer term survival rates are less than 2%.

Finding A Cure

Unfortunately, 200 cases per year is statistically insignificant in the battle for ever-shrinking funding for cancer research from the federal government. While all pediatric cancer funding is woefully inadequate, DIPG funding is nearly nonexistent. Most innovative DIPG research currently underway in the United States is privately funded, in many instances by foundations such as Hope For Caroline.

Additional financial support for DIPG research is desperately needed and could yield benefits far beyond those directly affected by this cruel disease. Because it is highly resistant to chemotherapy and affects children, whose treatment has historically led to innovations in other cancer treatments, DIPG presents an opportunity for all cancer research. Please consider a financial contribution to Hope For Caroline to support DIPG research.

Sources:

American Brain Tumor Association

http://www.abta.org/understanding-brain-tumors/

Boston Children's Hospital

http://www.childrenshospital.org/az/Site762/mainpageS762P0.html

The Childhood Brain Tumor Foundation

http://www.childhoodbraintumor.org/brain-tumor-types-and-imaging/57-brain-stem-gliomas-in-childhood.html

The Cure Starts Now Foundation

http://www.thecurestartsnow.org/about/brain-cancer/dipg/

St. Jude Children's Research Hospital

http://www.stjude.org/stjude/v/index.jsp?vgnextoid=b86c061585f70110VgnVCM1000001e0215acRCRD&vgnextchannel=bc4fbfe82e118010VgnVCM1000000e2015acRCRD

U.S. Food and Drug Administration