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H3 K27–Altered Diffuse Midline Glioma From Biopsy to Treatment: Neurosurgical Decisions in the Era of Molecularly Directed Therapy

Slideset

These downloadable slides on the neurosurgical management of H3 K27-altered diffuse midline glioma (DMG) focus on the role of stereotactic biopsy, molecular diagnosis, and treatment planning. Topics include evaluating the risks and benefits of tissue acquisition based on tumor location and clinical presentation; optimizing biopsy techniques and tissue stewardship to support integrated molecular diagnosis; and interpreting H3 K27M immunohistochemistry, H3K27me3 assessment, sequencing, and cerebrospinal fluid tumor DNA testing. The slides also review clinical evidence supporting dordaviprone in progressive H3 K27–mutant DMG, the ongoing phase III ACTION trial, and investigational approaches including ONC206 and CAR T-cell therapies targeting GD2 and B7-H3. Additional considerations include managing neurologic deterioration and hydrocephalus, coordinating multidisciplinary care, and optimizing referral timing and procedural planning to preserve treatment and clinical trial opportunities.

Released: October 31, 2026

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Faculty

Gerald A. Grant

Gerald A. Grant, MD, MBA

Allan H. Friedman Distinguished Professor of Neurosurgery
Chair, Department of Neurosurgery
Duke University School of Medicine
Durham, North Carolina
Sanghee Lim

Sanghee Lim, MD, PhD

Instructor of Neurology
Edward C. Kendall Fellow in Biochemistry
Clinician-Investigator Training Program
Division of Neuro-Oncology
Mayo Clinic
Rochester, Minnesota

Provided by

Provided by Clinical Care Options, LLC dba Decera Clinical Education.

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Supporters

This activity is supported by an educational grant from Jazz Pharmaceuticals, Inc.

Jazz Pharmaceuticals, Inc.

Target Audience

This activity is intended for neurosurgeons, pediatric neurosurgeons, neurosurgical oncologists, neurosurgical fellows, and residents who evaluate or manage patients with diffuse midline glioma. 

Learning Objectives

Upon completion of this activity, participants should be able to:

  • Determine when stereotactic biopsy is likely to meaningfully alter diagnosis, treatment eligibility, or clinical trial access in patients with suspected DMG, considering tumor location, procedural risk, neurologic status, and alternatives to tissue acquisition

  • Apply neurosurgical tissue-acquisition and specimen-stewardship strategies that support integrated diagnosis through coordinated use of H3 K27M IHC, H3K27me3 assessment, sequencing, and broader molecular testing, while recognizing the adjunctive role and current limitations of CSF-tDNA

  • Evaluate current evidence for systemic therapy in progressive H3 K27M-mutant DMG, including dordaviprone efficacy, safety, regulatory status, evidence limitations, and ongoing Phase III evidence generation, distinguishing approved use in progressive disease following prior therapy from investigational post-radiotherapy use in newly diagnosed disease

  • Coordinate neurosurgical management across the DMG continuum, including hydrocephalus and neurologic deterioration, consideration of repeat tissue or CSF acquisition, and timely referral for systemic, cellular, and locoregional clinical trials

Additional Disclosures

Generative artificial intelligence (AI) tools were used for data analysis or summarization influencing content and revising or refining educational content. All AI-assisted content underwent human review, editing, and validation by qualified faculty and accredited education staff to ensure scientific accuracy, balance, and compliance with the ACCME Standards for Integrity and Independence in Accredited Continuing Education.