DIPG is a tumour located in the pons (center) of the brain stem. Gliomas are a general term for tumours that arise from supporting tissue called glia, which help keep neurons in place and function properly. DIPG is brain stem glioma. The brainstem is the lowest part of the brain that connects the cerebrum to the spinal cord. The brainstem contains all the neurons that enter the spinal cord and the various structures which are involved in the sensation and control of throat and facial muscles and eye movements.
Diagnosis of DIPG
As with most brainstem tumours, the diagnosis of DIPG usually includes non-invasive brain imaging tests such as MRI in addition to neurological physical examination. Biopsy and other procedures are very rare. Like DIPG, diffuse median glioma often falls into the same category as DIPG for diagnosis and treatment, and is often grouped together. Recently, biopsies have been performed to select the best option for clinical trials. In connection with studies from the DIPG / DMG registry, and DIPG / DMG collaboration, statistics show that approximately 150-300 patients are diagnosed with DIPG annually in the United States, with a median age of DIPG patients of approximately. I’m 6-7 years old. The ratio of elderly to male / female with DIPG is 1: 1.
Treatment of Diffuse Intrinsic Pontine Glioma
Standard DIPG treatment is 6 weeks of radiation therapy, often with significant improvement in symptoms. However, the symptoms generally recur after 6-9 months and then progress quickly.
Neurosurgery to treat DIPG
Surgery to remove the tumour is usually not possible or recommended by DIPG. By nature, these tumours spread and invade the brainstem and grow between normal nerve cells. Aggressive surgery causes serious damage to leg and arm movements, swallowing, eye movements, breathing and even the neural structures essential to consciousness. Neurosurgery for immunotyping of diffuse endogenous pontine glioma Brainstem biopsy has recently played a limited role in experimental, therapeutic and clinical trials. However, this is not the current standard of care as it represents a significant risk from the biopsy site and is therefore properly performed only within the framework of participation in ongoing clinical treatment studies. Pontin biopsy is by no means a curative surgery and is only at risk (catastrophic and fatal) if the diagnosis is uncertain or if the patient is enrolled in an approved clinical trial.
Radiotherapy Treatment of DIPG
Traditional radiation therapy limited to the affected tumour area is the mainstay of treatment for DIPG. Previously, super split radiation therapy was used to deliver high doses of radiation, but did not improve survival. In the treatment of DIPG, radiosurgery is involved and in specific cases can be considered.
Chemotherapy Treatment of DIPG
The role of chemotherapy remains unclear but efforts are underway by the Pediatric Oncology Group (COG), the Pediatric Brain Tumor Consortium (PBTC), and others to further investigate the use of chemotherapy and other drugs, but studies have shown little improvement in survival. Drugs that enhance the effectiveness of radiation therapy have shown no additional benefits, but promising new active ingredients are being investigated. Beta interferon with immunotherapy and other drugs has also shown little effect in the study. Intensive or high-dose chemotherapy with autologous bone marrow transplantation or peripheral blood stem cell rescue has not been shown to be effective in brainstem glioma. Future clinical studies may include agents that disrupt cellular signalling pathways.
Prognosis of DIPG
A survival rate of less than 1% is seen under DIPG. The average overall survival for children diagnosed with DIPG is about 9 months. The 1 and 2 year survival rates are 30% to less than 10% thus making DIPG one of the most harmful childhood cancers.
Although 75-85% of patients show improvement in symptoms after radiation therapy, DIPGs begin to grow again. Clinical studies show the average time from radiation therapy to progression is 5 to 8.8 months. Patients whose tumours have begun to grow again go for experimental treatment in clinical trials to stop the growth of the tumour. However, studies have not shown significant benefits from the experimental DIPG therapy.
Last Modified: February 13, 2024