The diagnosis of diffuse intrinsic pontine glioma (DIPG) or diffuse midline glioma (DMG) is often casually regarded as a terminal cancer, but what are the true statistics regarding survival? For the first time since the classification of DIPG and DMG we now have these numbers due to the selfless participation of over 1,400 patients in the International DIPG/DMG Registry and the SIOPe (European) DIPG Registry. The largest linked database of DIPG and DMG data comprising the cooperative efforts of over 110 hospitals in 17 countries, it was one of the most ambitious and largest project created from the DIPG community after the 2011 DIPG Symposium. And now, it is starting to aid in the understanding of this “homerun” cancer that may provide clues to a cure for all cancers.
In short, there are DIPG survivors. Although typical overall survival ranges from 8-11 months, there are several characteristics that may lead to an improved diagnosis. These characteristics include those that are diagnosed either before the age of 3 or after the age of 10, those patients that have fewer symptoms at diagnosis, those patients that are diagnosed with a smaller tumor that does not extend beyond the pons and those patients that have tumors with an expression of a histone mutation. Considering all diagnoses, the 2-year survival rate typically is 10% and the 5-year survival rate is around 2%. Still considering that the majority of DIPG and DMG patients tend to be children, even these survival rates are difficult.
Where can I get more data on DIPG and DMG long term survivors?
Since 1996, there have been three survivorship studies that are typically regarded as primary sources of DIPG and DMG data. Each focuses on a different aspect of DIPG, from radiotherapy to commonalities of long term survivors. They can be found at:
- Long Term Survivors of Childhood Brain Stem Gliomas Treated with Hyperfractionated Radiotherapy
- Clinico-Radiologic Characteristics of Long-Term Survivors of Diffuse Instrinsic Pontine Glioma
- Clinical, Radiologic, Pathologic, and Molecular Characteristics of Long-Term Survivors of Diffuse Instrinsic Pontine Glioma (DIPG): A Collaborative Report From the International and European Society for Pediatric Oncology DIPG Registries
Generally each of these reports tend to confirm the limited, but potential survival from DIPG and DMG.
What are the traits of survivors of DIPG?
In 2017, an informal attempt was made to assess any patterns of survivors of DIPG and DMG. Through the efforts of The Cure Starts Now Foundation and the DIPG Collaborative, 14 long term survivors were anonymously brought together as part of the DIPG Symposium in a session labeled as "Club 22." During this effort discussions were held to review treatment paths, lifestyle choices and limited medical backgrounds in an attempt to find patterns not currently present in the registries. Unfortunately most confirmed the characteristics already defined of long term survivors in the registry report of 2018. Still it is important to note that as a result of the “Club 22” conference, nearly all survivors were registered in the International DIPG/DMG Registry.
Why can’t I find survivors of DIPG?
In most cases, long term survivors of DIPG and DMG fall victim to the stigma of being a survivor. Bias in the community that express that there “aren’t any survivors” or that survivors are likely “misdiagnosed” tend to lead to distrust and alienation from the DIPG community. For this reason, many survivors often do not engage in community functions or participate in awareness efforts. It is recommended that if we are to truly understand and benefit from their experience that the DIPG community develop specific survivorship sessions designed around their unique issues and quality of life difficulties.
What can I do to help?
If you want to change the statistics, please consider giving a donation towards homerun cancer research.