D I P G
Diffuse Intirensic Pontine Glioma
There are 120 different types of brain tumors, making it the second most common type of pediatric cancer. Sadly brain tumors are the leading cause of death in child with cancer. According to the Pediatric Brain Tumor Foundation, 9 children everyday in the US are diagnosed with a brain tumor.
What is DIPG ?
DIPG stands for Diffuse Intrinsic Pontine Glioma. It is a brain tumor found in the pons, a part of the middle of the brain stem. The pons is a vital area of the brain that controls important bodily functions such as breathing, heart rate, swallowing, speech, balance and eye movement.
The majority of brain stem tumors occur in the pons, diffusely infiltrate (they grow amidst the nervous), and cannot be surgically removed. Glioma is a general name for any tumor that arises from the supportive tissue called glia. Each year about 300 children in North America receive a devastating DIPG diagnoses.
The World Health Organization classifies brain tumors in grades 1-4 according to how fast they grow, how they tend to behave and if they are likely to spread. A grade1-2 tumor would be considered low grade and slow growing. Grade 3-4 tumors are fast growing and more aggressive.
What Causes DIPG ?
More research is still needed to find out what causes DIPG. Currently, there is no evidence that DIPG is caused by genetics or environmental factors. It most commonly occurs in children between the ages 5-10 year of age. During this time frame the brain goes through significant development and it is thought that this might be linked to the cause of DIPG. Although the majority of DIPG tumors are found in children, there are a few cases of adults and even newborn infants.
How is DIPG Treated ?
DIPG is one of the most challenging tumors to treat with a less than 1% chance of survival. Patients are diagnosed by their initial symptoms and MRI scans. In the last decade technology has advanced to enable neurosurgeons to successful biopsy DIPG tumors. Biopsies are risky because the pons is the most important area of the brain controlling all vital bodily functions. Biopsies provide genetic information and mutations found in each DIPG tumor. Hopefully in the near future, researchers will be able to find the right medications to target the specific mutations found in DIPG.
Surgery to remove a DIPG tumor is impossible because of its diffuse nature. It is not a solid tumor but rather mixed in with healthy brain cells. Radiation directly to the brain is considered the standard of care and this has not changed in over three decades. With radiation, the average length of survival is 9 months. Radiation treatments are given 5 days a week for 6 weeks. The effects of radiation only pause or temporarily shrink the tumor for approximately 70% of patients with DIPG. The other 30% of DIPG patients do not receive any symptom relief and can actually have an increase in symptoms from pseudo progression caused by inflammation due to the radiation treatments.
Despite DIPG patients participating in hundreds of clinical trials, no drug(s) have been proven to increase the average length of survival. Because new pediatric DIPG trials take years to get governmental approval, families who want to fight have to turn to experiment treatments that are given on a compassion bases and have to be self funded.