DIPG (diffuse intrinsic pontine glioma) is the most common brainstem tumour found in children, a highly sensitive portion of the brain vital for human beings. As with the majority of growth tumours, it is not known why these malignant cells specifically invade the brainstem so diffusely. It normally occurs in children between 5 and 10 years of age. The symptoms are fairly characteristic, consisting of alterations in facial movement, difficulty in swallowing and walking, and weakness in the arms or legs. These symptoms commonly develop within a few weeks. Unfortunately, there is no curative treatment for this disease.
Our data
Diagnostic approach
DIPG is a disease that is diagnosed on the basis of the typical symptoms described above and from well-defined radiological criteria in an MRI scan. To this day, a biopsy has proved essential for confirming diagnosis. However, centres of reference such as the SJD Barcelona Children's Hospital, which are engaged in research projects and/or clinical trials where the analysis of tumour tissue is essential, value performing this procedure at the time of diagnosis.
A biopsy usually confirms the existence of a glial-type tumour. Molecular analysis of these tumours has revealed that in the majority (80% of cases) a mutation of the K27M gene is identified in two variants, the H3.3 (60% of cases) and the H3.1 (20% of cases). These molecular changes, in combination with other genetic alterations (e.g. p53, ACVR1, PDGFR), are fundamental in the onset, development and progression of the disease.
Treatment and research of DIPG
Therapeutic approach
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Carrying out biopsies in diagnosed patients
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Molecular study of cases
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Clinical immunotherapy trial
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Research at SJD Barcelona and worldwide
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Participation of families in the research
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