Hardly anyone has ever heard of DIPG. This abbreviation stands for Diffuse Intrinsic Pontine Glioma and describes a childhood brain tumor. There’s no cure or positive life-changing treatment at the moment. Not much has happend in the research in the last years. The foundation for innovative medicine in Munich is now fighting against this rare disease.
Tumors of the central nervous system
In Germany every year about 1,750 children youger than 15 years getting the diagnosis cancer, another 360 children between the age of 15 and 17.1 Tumors of the central nervous system (CNS) are the second leading types of cancer after leukemia among children with a incidence of 430 kids per year.1,2
In contrast to the peripheral nervous system, the central nervous system includes the nerve tracts of the brain and spinal cord.3 It serves as a central organ regarding integration, coordination and regulation and processes both external sensory impressions and interior stimuli.3 A major problem in the treatment of brain tumors is the blood-brain barrier, which separates the brain and spinal cord from the bloodstream.4,5 This barrier ensures that the blood vessels in the brain are protected from harmful substances, which are possibly present in the bloodstream.4,5 Thus, not all cytotoxic drugs are able to overcome this barrier.4,5
Gliomas are defined as solid tumors of the central nervous system caused by degeneration of glial cells, the supporting tissue of nerve cells.2,6 They are classified into low-grade and high-grade gliomas.6
Among children and young people high-grade gliomas rarely occur with a proportion of 10-15% of all tumors of the central nervous system.6 About 60-80 kids and adolescents under the age of 15 get the diagnosis of a high-grade glioma in Germany every year.6 This corresponds to a proportion of 5-10 patients per 1,000,000 children.6 High-grade tumors grow rapidly and aggressively, destroy healthy tissue and can contribute to the formation of new tumors through migration.6
Depending on the location, origin and malignancy of gliomas, different forms can be distinguished.6 The variant with the worst prognosis is the Diffuse Intrinsic Pontine Glioma.6
Diffuse Intrinsic Pontine Glioma (DIPG)
Diffuse Intrinsic Pontine Glioma describes a tumor which is located in the brain stem and is responsible for one-third of all high-grade gliomas among children.6 The brain stem includes the midbrain, pons and the Medulla oblongata. It controls vital bodily fuctions such as breathing, the blood pressure and reflexes. This area is the central junction of all nerves running between the brain and the rest of the body.6
DIPG is also called Diffuse Midline Glioma with Histone H3K27M Mutation,6 because in 78% of all cases histone H3 is non-functional by changing a lysine (K) with a methionine (M) at position 27 through a point mutation.7 Histones are important for both DNA packaging and gene expression. If these proteins are mutated, changes in the expression of certain genes override essential control mechanisms, which in turn promotes tumor development.7
Due to the localization of the pontine glioma and the diffuse infiltrating grwoth, this malignant tumor is inoperable and there’s no chance of healing.8
DIPG is a rare disease with a total of 300 new cases in Europe and North America each year.8 Nevertheless, DIPG is the most common fatal brain tumor among children (between the age of 4 and 9 years). The 5-year survival rate is less than 1%.8 After getting the diagnosis, the median survival rate is 9 months.8
The current standard therapy is radiation, there’s no effective chemotherapy.9
Foundation for innovative medicine
The foundation for innovative medicine, which was founded in 2014 and consists of a volunteer team, has focused on the research and prevention of cancer, especially on childhood brain tumors.10 The perform for example projects helping to define effective therapies for incurable cancer types.10
A private initiative called DIPG Fighter was originated after the death of Lina (7 years). Their aim is supporting the foundation financially regarding DIPG research. Currently, a new technology is developed, which will allow predictions about the chances of drugs crossing the blood-brain barrier. This should speed up finding a appropriate treatment for DIPG.
There’re many activities started by the DIPG Fighter to raise funds for this enormously important research.
Kristina Schraml (email@example.com)