"Diffuse Intrinsic Pontine Glioma" - a cruel disease. Though rare, affecting mostly children, DIPG is the cruelest and most virulent of all possible brain stem tumors. The number of cases arising in the USA in any year is less than 200. It affects children mostly between ages 5 and 10. Because it is so entwined in the brain stem, the tumor is inoperable. Its growth is unrelenting and, so far, ultimately unstoppable.
The name says it all:
"Diffuse" — it is spread among the nerves and tissues of the brain stem.
"Intrinsic" -- it is wrapped amongst them, making surgery or accurate and selective radiation virtually impossible.
"Pontine" -- it is located in the pons (middle) of the brain stem.
"Glioma" -- the tumor arises from the glia, the tissues that support the neurons (the thinking cells).
The brain stem is the lowest section of the brain, transmitting signals from the brain to the spinal cord and converting instructions from the brain to the motors of the body. Tumors in the brainstem can be located in the cervicomedullary junction, the midbrain, the tectum or the pons. The pons is the most difficult to reach.
While radiation therapy can shrink the tumor and bring a temporary respite, an irremediable regression follows. The median survival rate is nine months.
St Jude's Hospital in Tennessee, an institution leading research in this field, sums up the current desperate lack of knowledge: "In their quest for a cure, DIPG children must move from one experimental protocol to another, enduring treatments with side-effects unacceptable with any other diagnosis. The cruelty of this disease cannot be denied. Sparing their cognitive abilities, DIPG slowly robs children of their motor functions resulting in partial paralysis, loss of voice and sight and finally ending with an inability to eat and breathe. It is both heart wrenching and painful as they are fully aware of their decline often until their last day." We currently DO NOT know what causes brain tumors. The major causes seem to be chromosomal and genetic abnormalities. Research so far has statistically proved very few instances of possible environmental causes for childhood cancer. The Children's Oncology Group (COG) continues to conduct epidemiology, cytogenetic, and microbiology studies in their quest for answers. Meanwhile our Foundation does its best for victims of this and other incurable childhood diseases, and for the families who are devastated.