Researchers do not know yet exactly what causes DIPG. Like most cancers, DIPG occurs when something goes wrong with process of cell reproduction.

Cells use DNA, the building blocks of genes, in order to carry out various functions, including making more cells. Sometimes pieces of DNA become damaged or are copied incorrectly. Scientists call this a mutation. Usually, cells with mutations in their DNA either repair the mutation or die (a process called apoptosis). Sometimes, though, cells with mutations can’t be repaired and don’t die like they are supposed to. In certain cases, these mutated cells begin to divide quickly, which may cause a person to develop cancer.

In recent years our understanding of the biology of DIPG has advanced considerably with the increase in the availability of tumor specimens. Researchers have begun to understand the exact genetic mutations involved with DIPG through the use of genetic sequencing. They have discovered the most common mutations involved in DIPG, and are now working to better understand these mutations, in order to engineer new drugs or repurpose preexisting drugs that will be able to effectively treat DIPG.

Currently, there is no evidence that DIPG is caused by any environmental factor, such as exposure to chemicals or radiation. There is also no evidence that specific inherited genetic variations contribute to DIPG.