Treatment of PGM can entail either surgical resection or targeted radiation therapy. Survival rates in the former subtype are significantly worse but are similar to those of the latter subtype.
Patients with diffuse intrinsic pontine glioma demonstrate a wide range of neuroimaging abnormalities with no clear characteristic. As of 2013 the underlying cause for PPG is still largely unknown. The presence of T2 weighted lesions in the pons in a subset of PPG cases may indicate a relationship to diffuse pontine astrocytoma in some patients.
There are a number of common treatments for patients with diffuse intrinsic pontine gliomas, including surgery (total resection or subtotal resection with radiotherapy), chemotherapy, and radiation therapy. There are no trials that evaluate the use of adjuvant therapy in the management of patients with diffuse intrinsic pontine gliomas. Most often, surgery and radiation therapy are administered as part of multimodality therapy.\n\nWhile the majority of patients will receive a palliative treatment, several patients will receive active ongoing treatment. These patients are considered candidates for a "maintenance palliative treatment" of some active modality (chemotherapy, radiation therapy, or surgery).
Symptoms of diffuse intradural glioma may include motor deficits, ocular gaze instability, nystagmus, dysarthria and unilateral hearing loss. There may also be signs of supratentorial intracranial compression. In some cases, patients have symptoms even without the presence of a tumor and therefore, patients have to be assessed for other causes. DOPG may be mistaken for migraine, encephalitis, or depression. Neurocognitive deficits, especially in executive function, may serve as a biomarker for diagnosis of the disease.
the current estimate is around 50,000 people a year have DAIG; this may represent half the people with PPA/DIPG who have no family history. The proportion of patients tested in the USA that have tested positive for BRAF and ERBB2 mutations, which are associated with higher risk of progression or shorter survival, will be important in determining whether this is a bona fide disease entity or a subtype of PPA/DIPG on the spectrum of PPL/DAIPG.
This disorder is a form of gliomas involving mainly diffuse involvement of the corpus callosum and the superior and posterior parts of the third ventricle. These tumors produce symptoms ranging from headache and weakness, to hemiplegia, apraxia, and nystagmus. Imaging studies may reveal periaqueductal or ventricular involvement and periaqueductal mass. There are no histological differences between diffuse intrinsic pontine gliomas and brainstem gliomas. Diffuse intrinsic pontine gliomas are treated with surgical resection, radiation, and chemotherapy. Patients with progressive neurological impairment are treated with chemotherapy and radiotherapy.
Despite the fact that the majority of patients are nonsmokers, our data suggest that smoking may be an important risk factor in patients with diffuse intrinsic pontine glioma. Further investigation is warranted regarding the possible interaction between smoking and IDH1 R132H mutations in these patients.
Patients receiving hypofractionated therapy on a combined schedule with conventional chemo/radiation are less likely to receive total radiation dose <25 Gy or to receive no chemotherapy. This should be considered for clinical practice as well as future prospective study.
• DIPG has a dismal prognosis with a median overall survival of 12.3 months and disease-specific survival of 7 months. • Age at diagnosis of DIPG and younger age and male gender are both risk factors for DIPG-associated survival and disease-specific survival.
The majority of adults diagnosed with diffuse intrinsic pontine glioma developed their disease after age 65. This suggests that while diffuse intrinsic pontine glioma is a late onset disease in most patients, it represents an under-recognized disease in adults.
Based on previous reports using 1.8-Gy fractionation and a very high RT-CBR, it is possible that the risk of acute and late toxicity may be higher for HIFN than for EF or RT alone.
Both radiation treatment modalities were associated with improvements in quality of life. Results from a recent paper may be explained by the reduction of symptoms in a high proportion of patients. Further improvement occurred after the initial course of post-relapse chemotherapy but not after the initial post-reimplant radiotherapy. This article is protected by copyright. All rights reserved.