Bridging Community and Academic Practice: Multidisciplinary Approaches to Diffuse Midline Gliomas - Episode 1
H3K27M-Mutant DMG: Early Symptoms and Imaging Pathway
Experts discuss the clinical complexity of diffuse midline glioma with H3K27M mutation, emphasizing how symptom patterns vary by tumor location—impacting motor, language, or visual function—and highlighting the importance of prompt neuroimaging and multidisciplinary management for early diagnosis and treatment planning.
Funding support provided by Chimerix/Jazz Pharmaceuticals. Content independently developed and published by OncLive.
Diffuse midline glioma (DMG) with the H3K27M mutation is an aggressive central nervous system tumor, most commonly affecting children and adolescents, though it can also present in adults. The clinical presentation varies significantly depending on the tumor's anatomical location. When the tumor is situated near motor pathways—such as the corona radiata or internal capsule—patients often exhibit motor deficits, ranging from subtle weakness to more pronounced motor impairment. If the lesion involves subcortical language pathways, particularly in the dominant hemisphere, language disturbances such as difficulties with comprehension or expression may occur. Tumors located near the visual pathways can lead to visual field deficits, typically affecting the contralateral side.
In addition to focal neurological symptoms, diffuse midline gliomas can cause increased intracranial pressure due to obstructive hydrocephalus. This is often the result of mass effect or compression of the ventricular system and drainage pathways, especially when the tumor is located in or near midline structures such as the thalamus, brainstem, or spinal cord. Symptoms of increased intracranial pressure can include headaches, nausea, vomiting, and altered mental status, which may prompt urgent evaluation. The constellation of symptoms, while variable, provides critical clues for timely diagnosis and management.
Neuroimaging plays a central role in the initial assessment. Once a patient presents with neurological symptoms that localize to a specific region of the nervous system, prompt imaging is typically initiated. A thorough clinical history helps determine the timeline of symptom progression, allowing clinicians to assess the acuity and severity of the presentation. MRI remains the gold standard for evaluating suspected gliomas and is essential for identifying tumor location, size, and characteristics. Early identification through imaging is vital to developing a multidisciplinary treatment strategy aimed at symptom control and disease stabilization.
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