Diffuse Intrinsic Pontine Glioma

2010-10-07

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Diffuse intrinsic pontine glioma is a tumor located at the lowest part of the brain at the brain stem. It can be benign or cancerous and it affects children. The average age for diagnosis of the disease is between 5 and 9 years old. Tumors develop from the glial, the supportive tissue that joins brain matter together. The tumors and are fast growing and are usually diagnosed within one month from the onset of symptoms. There does not seem to be any predisposition to the disease.

Symptoms

Symptoms can vary per patient. The most common include:

difficulty walking, chewing, and swallowing.
facial expressions and eye movements may be impaired.
a weakness can occur in the arms and legs.
as the increased pressure builds in the brain hydrocephalus will develop from the fluid build up.
headache will occur while awakening as well as vomiting and fatigue.

Any of these symptoms can be signs of several diseases. It is important to see a doctor as soon as any symptom occurs.

Diagnosis

Professionals rely on modern technology to diagnose this type of glioma. The CT scan with contrast (injection dye) infiltrates the tumor giving a clear picture of the size and density. Tumors usually appear as round masses that cause the pons to expand.

MRI, a procedure that uses magnets and radiofrequencies, can distinguish between tumor and normal tissue better than a CT scan. Contrast is used that can show growth in vivid sections of the brainstem.

An MRS, magnetic resonance spectroscopy is a test used with MRI that can detect organic compounds produced by the body’s metabolism. It can distinguish between a tumor that is neuronal (rare but has a good cure rate) or gliomal.

Treatment

Treatments are determined by the child’s age, health, tolerance to medication, size and location of the tumor, prognosis and the choice of the parents.

Surgery is not an option with diffuse intrinsic pontine glioma because the complexity of the tumors that grows around the brain stem. According to the University of Pennsylvania Hospital only 10 percent of children with the disease will live past eighteen months of diagnosis.

Radiation is used as the choice of treatment for the disease. Studies that increase the current dose of radiation over time have not proven to increase the chances of survival. Chemotherapy drugs have not proven affective though research continues in the use of new chemotherapy drugs.

Recurrence of the disease and continued growth of the tumor will usually occur within a year of the initial treatment. No other treatment has been successful

Research

Two research studies that began in January 2010 are St. Jude’s Hospital and the Children’s Hospital Medical Center in Maryland.

St. Jude’s Hospital is conducting research using a combination of Vandetanib and Dasatinib, two drugs used in the treatment of other cancers. The combination drugs will be used during and after radiation treatment.

The Children’s Hospital Medical Center in Maryland is using Avastin as a trial treatment. Avastin has been used in the treatment of metastatic cancers such as colon and breast cancer. Both studies will run for several years.

Where To Turn

When parents are overwhelmed by the suffering of their child from this dreadful disease they can turn to organizations that can help them cope. The following is a list of organizations that can offer support and news on the latest research.

Reflections of Grace Foundation : https://reflectionsofgrace.org/index.php/diffuse-intrinsic-pontine-glioma-dipg.html
The Cure Starts Now Foundation : https://www.thecurestartsnow.org/believe/
The Childhood Brain Tumor Foundation: https://www.childhoodbraintumor.org/index.php/links/84-diffuse-intrinsic-pontine-glioma.html

Summary

If your child is suffering from this disease find a support group of parents that are experiencing the same things that you are. Learning to cope with the ups and downs of treatment can be very stressful. Parents can find strength from another.