Diffuse midline glioma

[4][5] Currently, the standard of care is fractionated external beam radiotherapy, as the tumour location precludes surgery, and chemotherapy has shown to be ineffective.

[9] Like most brainstem tumors, diagnosing diffuse intrinsic pontine glioma usually involves non-invasive brain imaging like MRI, in addition to neurologic physical exam.

A neurosurgically performed brainstem biopsy for immunotyping of diffuse intrinsic pontine glioma has served a limited recent role in experimental clinical studies and treatment trials.

This, however, is not the current standard of care, as it presents considerable risk given the biopsy location, and thus is appropriately performed only in the context of participation in an ongoing clinical treatment trial.

Pontine biopsy is in no way a therapeutic or curative surgery, and the risks (potentially catastrophic and fatal) are only outweighed when the diagnosis is uncertain (extremely unusual) or the patient is enrolled in an approved clinical trial.

Studies have shown little improvement in survival, although efforts (see below) through the Children's Oncology Group (COG), Paediatric Brain Tumour Consortium (PBTC), and others are underway to explore further the use of chemotherapy and other drugs.

Neoepitope specific peptide vaccines targeting the clonal driver mutation H3 K27M have been shown to elicit cytotoxic T-cell and T-helper cell responses in patients with diffuse midline glioma.

Future clinical trials may involve medicines designed to interfere with cellular pathways (signal transfer inhibitors), or other approaches that alter the tumor or its environment.

[18] Although 75–85% of patients show some improvement in their symptoms after radiation therapy, DIPGs almost always begin to grow again (called recurrence, relapse, or progression).

Researchers and clinicians have tried several methods to overcome the blood–brain barrier: The definitive genetic marker of a diffuse midline glioma is H3K27me3 loss.

Diffuse midline gliomas have three known subtypes:[25] Notes Left Behind, a non-fictional book published in 2009, is about a girl named Elena Desserich.

Biopsy sample from a diffuse intrinsic pontine glioma.
Magnetic resonance spectroscopy of a diffuse intrinsic pontine glioma showing elevated choline and creatine peaks with a decreased NAA peak.
Radiotherapy for a young adult patient with a diffuse intrinsic pontine glioma. Color indicates radiation dose .
Summary of a meta analysis of over 1,000 cases of DIPG and high-grade pediatric gliomas, highlighting the mutations involved as well as generic outcome information.
Mutations in diffuse intrinsic pontine glioma samples from several anatomical locations.
Schematic of a currently experimental approach to DIPG drug delivery involving nanoparticles and stem cells .