Diffuse intrinsic pons glioma

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The diffuse intrinsic pontine glioma (short DIPG, according to WHO classification as "diffuse midline glioma, H3 K27M-mutant") which is a brain tumor, which particularly occurs in childhood and in the brainstem , thalamus and spinal cord may be located.

It is the most common fatal brain tumor in childhood, although it is comparatively rare, accounting for 10–15% of all pediatric brain tumors (corresponds to around 300 cases per year in Europe and North America). As a glioma , DIPG originates from the glial cells, the supporting tissue of the nerve cells. Due to the typical location in the brain stem ( pons ) and the infiltrating growth, this highly malignant tumor is inoperable and the prospects for a cure are currently minimal, with an average three-year survival rate of 3%, and less than 1% for five years. After diagnosis, the median survival time is nine months.

The current standard therapy is radiation. No effective chemotherapy has yet been established. More recent studies suggest that histone deacetylase (HDAC) inhibitors may be effective. A further characterization of the tumor and its therapy are still the subject of research, which is carried out by international institutions and foundations such as For example, the Dana-Farber Cancer Institute in Boston or the Georg-August University in Göttingen are dedicated to this.

The symptoms are varied and can appear extremely quickly and in different degrees. The most common symptoms include headache and / or back pain, dizziness, loss of appetite, nausea and vomiting (in the case of a brain tumor, typically independent of food intake [vomiting] and often in the morning and when lying down), weight loss, increasing tiredness, poor performance, concentration disorders and changes in personality. Those affected are not mentally restricted and fully conscious.

Individual evidence

  1. ^ WHO classification 2016 - The classification of tumors of the CNS. January 1, 2016, accessed November 6, 2018 .
  2. ↑ Children's cancer info - prognosis: What are the survival prospects of patients with highly malignant glioma. July 9, 2014, accessed June 20, 2018 .
  3. ↑ Contemporary survival endpoints: study on International Diffuse Intrinsic Pontine Glioma Registry. July 31, 2017, accessed November 6, 2018 .
  4. ↑ Mikey Czech Foundation donates USD 1 million for DIPG research at Dana-Farber Cancer Institute. March 30, 2017. Retrieved November 6, 2018 .
  5. ↑ Karremann M, Krämer N, Hoffmann M, Wiese M, Beilken A, Corbacioglu S, Dilloo D, Driever PH, Scheurlen W, Kulozik A, Gielen GH, von Bueren AO, Dürken M, Kramm CM .: Haematological malignancies following temozolomide or pediatric high-grade glioma. Eur J Cancer. 2017 Jun 3; 81: 1-8.
  6. ↑ Maria Yiallouros: Highly malignant gliomas (brief information). Retrieved February 4, 2019 .