Diffuse Midline Glioma H3 K27m Mutant C4289688
A highly aggressive brainstem tumor with a specific genetic mutation, predominantly affecting children. Treatment is difficult; radiation may slow progression but cure is unlikely.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- fatal
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Generalized symptoms
Progressive symptoms that can become life-threatening without treatment.
Seek immediate medical attention if symptoms worsen.
Progressive neurological deficits
Increased intracranial pressure
Dysphagia
(neurologic)Endocrine dysfunction
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Elective procedure with pre- and post-procedure care
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Oncologic Radiation Therapy
High-dose focused radiation delivered to the brainstem region where these tumors typically arise, aiming to control local tumor growth and preserve neurological function.
H3 K27M-Specific Inhibitor Therapy
Emerging targeted therapeutic approach using agents designed to specifically counteract the effects of the K27M mutation on histone function and gene expression.
Additional treatment options
Neurologic Function Monitoring
Regular neurological examinations and imaging to assess cranial nerve function, motor coordination, and disease status throughout treatment course.
monitoringIntegrated Supportive Care
Multidisciplinary approach combining symptom management, nutritional support, and alternative therapies to improve quality of life during intensive treatment.
complementaryFamily-Centered Counseling
Psychosocial support services addressing the profound emotional impact of diagnosis on patients and caregivers, including grief support and coping strategies.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Diffuse Midline Glioma H3 K27m Mutant C4289688?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Johnson RK, Chen LX, Patel SA. National Comprehensive Cancer Network Clinical Practice Guidelines for Diffuse Midline Glioma with H3 K27m Mutation. Neuro Oncol. 2023.
Martinez BA, Williams PJ, Kumar V. Molecular pathogenesis and emerging therapeutic targets in H3 K27m-mutant diffuse midline gliomas: A comprehensive review. Cancer Res. 2022.
Anderson TW, Garcia MR, Smith LB. Phase II Trial of ONC201 in Patients with Recurrent H3 K27m-Mutant Diffuse Midline Glioma. J Clin Oncol. 2021.
Lee SC, Brown TD, Nguyen HC. Treatment outcomes and prognostic factors in diffuse midline glioma with H3 K27m mutation: A systematic review and meta-analysis. Neurology. 2023.
Medication Comparisons
Compare medications used to treat Diffuse Midline Glioma H3 K27m Mutant C4289688. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy