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Diffuse midline glioma, H3 K27-altered

MedGen UID:
1811313
•Concept ID:
C5669877
•
Neoplastic Process
Synonym: Diffuse Midline Glioma, H3 K27-Altered

Definition

A diffuse midline glioma characterized by H3 K27 alteration and usually either a histone H3 K27M mutation, an EGFR mutation, or aberrant overexpression of EZHIP. The prognosis is poor. [from NCI]

Professional guidelines

PubMed

H3 K27M-altered glioma and diffuse intrinsic pontine glioma: Semi-systematic review of treatment landscape and future directions.
van den Bent M, Saratsis AM, Geurts M, Franceschi E
Neuro Oncol 2024 May 3;26(Supplement_2):S110-S124. doi: 10.1093/neuonc/noad220. PMID: 38102230Free PMC Article
Pharmaco-proteogenomic profiling of pediatric diffuse midline glioma to inform future treatment strategies.
Findlay IJ, De Iuliis GN, Duchatel RJ, Jackson ER, Vitanza NA, Cain JE, Waszak SM, Dun MD
Oncogene 2022 Jan;41(4):461-475. Epub 2021 Nov 10 doi: 10.1038/s41388-021-02102-y. PMID: 34759345Free PMC Article

Recent clinical studies

Etiology

Characteristics of H3K27M-mutant diffuse gliomas with a non-midline location.
Guidara S, Seyve A, Poncet D, Leonce C, Bringuier PP, McLeer A, Sturm D, Cartalat S, Picart T, Ferrari A, Hench J, Frank S, Meyronet D, Ducray F, Barritault M
J Neurooncol 2024 Sep;169(2):391-398. Epub 2024 Jun 27 doi: 10.1007/s11060-024-04733-z. PMID: 38937309
Selective DRD2 antagonist and ClpP agonist ONC201 in a recurrent non-midline H3 K27M-mutant glioma cohort.
Odia Y, Hall MD, Cloughesy TF, Wen PY, Arrillaga-Romany I, Daghistani D, Mehta MP, Tarapore RS, Ramage SC, Allen JE
Neuro Oncol 2024 May 3;26(Supplement_2):S165-S172. doi: 10.1093/neuonc/noae021. PMID: 38386699Free PMC Article
Distinct MRI characteristics of spinal cord diffuse midline glioma, H3 K27-altered in comparison to spinal cord glioma without H3 K27-alteration and demyelination disorder.
Ying Y, Liu X, Li X, Mei N, Ruan Z, Lu Y, Yin B
Acta Radiol 2024 Mar;65(3):284-293. Epub 2023 Dec 20 doi: 10.1177/02841851231215803. PMID: 38115811
Development and validation of a machine learning algorithm for predicting diffuse midline glioma, H3 K27-altered, H3 K27 wild-type high-grade glioma, and primary CNS lymphoma of the brain midline in adults.
Lv K, Chen H, Cao X, Du P, Chen J, Liu X, Zhu L, Geng D, Zhang J
J Neurosurg 2023 Aug 1;139(2):393-401. Epub 2022 Dec 23 doi: 10.3171/2022.11.JNS221544. PMID: 36681946
MRI comparative study of diffuse midline glioma, H3 K27-altered and glioma in the midline without H3 K27-altered.
Zhao JP, Liu XJ, Lin HZ, Cui CX, Yue YJ, Gao S, Xu HZ
BMC Neurol 2022 Dec 22;22(1):498. doi: 10.1186/s12883-022-03026-0. PMID: 36550486Free PMC Article

Diagnosis

Characteristics of H3K27M-mutant diffuse gliomas with a non-midline location.
Guidara S, Seyve A, Poncet D, Leonce C, Bringuier PP, McLeer A, Sturm D, Cartalat S, Picart T, Ferrari A, Hench J, Frank S, Meyronet D, Ducray F, Barritault M
J Neurooncol 2024 Sep;169(2):391-398. Epub 2024 Jun 27 doi: 10.1007/s11060-024-04733-z. PMID: 38937309
Selective DRD2 antagonist and ClpP agonist ONC201 in a recurrent non-midline H3 K27M-mutant glioma cohort.
Odia Y, Hall MD, Cloughesy TF, Wen PY, Arrillaga-Romany I, Daghistani D, Mehta MP, Tarapore RS, Ramage SC, Allen JE
Neuro Oncol 2024 May 3;26(Supplement_2):S165-S172. doi: 10.1093/neuonc/noae021. PMID: 38386699Free PMC Article
A review of current therapeutics targeting the mitochondrial protease ClpP in diffuse midline glioma, H3 K27-altered.
Jackson ER, Persson ML, Fish CJ, Findlay IJ, Mueller S, Nazarian J, Hulleman E, van der Lugt J, Duchatel RJ, Dun MD
Neuro Oncol 2024 May 3;26(Supplement_2):S136-S154. doi: 10.1093/neuonc/noad144. PMID: 37589388Free PMC Article
Paediatric-type diffuse high-grade gliomas in the 5th CNS WHO Classification.
Gianno F, Giovannoni I, Cafferata B, Diomedi-Camassei F, Minasi S, Barresi S, Buttarelli FR, Alesi V, Cardoni A, Antonelli M, Puggioni C, Colafati GS, Carai A, Vinci M, Mastronuzzi A, Miele E, Alaggio R, Giangaspero F, Rossi S
Pathologica 2022 Dec;114(6):422-435. doi: 10.32074/1591-951X-830. PMID: 36534421Free PMC Article
Imaging features associated with H3 K27-altered and H3 G34-mutant gliomas: a narrative systematic review.
Lasocki A, Abdalla G, Chow G, Thust SC
Cancer Imaging 2022 Nov 17;22(1):63. doi: 10.1186/s40644-022-00500-3. PMID: 36397143Free PMC Article

Therapy

Selective DRD2 antagonist and ClpP agonist ONC201 in a recurrent non-midline H3 K27M-mutant glioma cohort.
Odia Y, Hall MD, Cloughesy TF, Wen PY, Arrillaga-Romany I, Daghistani D, Mehta MP, Tarapore RS, Ramage SC, Allen JE
Neuro Oncol 2024 May 3;26(Supplement_2):S165-S172. doi: 10.1093/neuonc/noae021. PMID: 38386699Free PMC Article
A review of current therapeutics targeting the mitochondrial protease ClpP in diffuse midline glioma, H3 K27-altered.
Jackson ER, Persson ML, Fish CJ, Findlay IJ, Mueller S, Nazarian J, Hulleman E, van der Lugt J, Duchatel RJ, Dun MD
Neuro Oncol 2024 May 3;26(Supplement_2):S136-S154. doi: 10.1093/neuonc/noad144. PMID: 37589388Free PMC Article
Development and validation of a machine learning algorithm for predicting diffuse midline glioma, H3 K27-altered, H3 K27 wild-type high-grade glioma, and primary CNS lymphoma of the brain midline in adults.
Lv K, Chen H, Cao X, Du P, Chen J, Liu X, Zhu L, Geng D, Zhang J
J Neurosurg 2023 Aug 1;139(2):393-401. Epub 2022 Dec 23 doi: 10.3171/2022.11.JNS221544. PMID: 36681946

Prognosis

Transcriptomic and proteomic spatial profiling of pediatric and adult diffuse midline glioma H3 K27-Altered.
Damodharan S, Shireman JM, Xie E, Distler E, Kendziorski C, Dey M
Sci Rep 2024 Sep 30;14(1):22668. doi: 10.1038/s41598-024-73199-w. PMID: 39349581Free PMC Article
Characteristics of H3K27M-mutant diffuse gliomas with a non-midline location.
Guidara S, Seyve A, Poncet D, Leonce C, Bringuier PP, McLeer A, Sturm D, Cartalat S, Picart T, Ferrari A, Hench J, Frank S, Meyronet D, Ducray F, Barritault M
J Neurooncol 2024 Sep;169(2):391-398. Epub 2024 Jun 27 doi: 10.1007/s11060-024-04733-z. PMID: 38937309
Selective DRD2 antagonist and ClpP agonist ONC201 in a recurrent non-midline H3 K27M-mutant glioma cohort.
Odia Y, Hall MD, Cloughesy TF, Wen PY, Arrillaga-Romany I, Daghistani D, Mehta MP, Tarapore RS, Ramage SC, Allen JE
Neuro Oncol 2024 May 3;26(Supplement_2):S165-S172. doi: 10.1093/neuonc/noae021. PMID: 38386699Free PMC Article
Development and validation of a machine learning algorithm for predicting diffuse midline glioma, H3 K27-altered, H3 K27 wild-type high-grade glioma, and primary CNS lymphoma of the brain midline in adults.
Lv K, Chen H, Cao X, Du P, Chen J, Liu X, Zhu L, Geng D, Zhang J
J Neurosurg 2023 Aug 1;139(2):393-401. Epub 2022 Dec 23 doi: 10.3171/2022.11.JNS221544. PMID: 36681946
Imaging features associated with H3 K27-altered and H3 G34-mutant gliomas: a narrative systematic review.
Lasocki A, Abdalla G, Chow G, Thust SC
Cancer Imaging 2022 Nov 17;22(1):63. doi: 10.1186/s40644-022-00500-3. PMID: 36397143Free PMC Article

Clinical prediction guides

Genetic alteration analysis of non-pediatric diffuse midline glioma, H3 K27-altered.
Jang H, Moon S, Kwon HJ, Lee S, Choe G, Lee KS
Hum Pathol 2024 Dec;154:105709. Epub 2024 Dec 17 doi: 10.1016/j.humpath.2024.105709. PMID: 39701425
Characteristics of H3K27M-mutant diffuse gliomas with a non-midline location.
Guidara S, Seyve A, Poncet D, Leonce C, Bringuier PP, McLeer A, Sturm D, Cartalat S, Picart T, Ferrari A, Hench J, Frank S, Meyronet D, Ducray F, Barritault M
J Neurooncol 2024 Sep;169(2):391-398. Epub 2024 Jun 27 doi: 10.1007/s11060-024-04733-z. PMID: 38937309
Selective DRD2 antagonist and ClpP agonist ONC201 in a recurrent non-midline H3 K27M-mutant glioma cohort.
Odia Y, Hall MD, Cloughesy TF, Wen PY, Arrillaga-Romany I, Daghistani D, Mehta MP, Tarapore RS, Ramage SC, Allen JE
Neuro Oncol 2024 May 3;26(Supplement_2):S165-S172. doi: 10.1093/neuonc/noae021. PMID: 38386699Free PMC Article
MRI comparative study of diffuse midline glioma, H3 K27-altered and glioma in the midline without H3 K27-altered.
Zhao JP, Liu XJ, Lin HZ, Cui CX, Yue YJ, Gao S, Xu HZ
BMC Neurol 2022 Dec 22;22(1):498. doi: 10.1186/s12883-022-03026-0. PMID: 36550486Free PMC Article
Imaging features associated with H3 K27-altered and H3 G34-mutant gliomas: a narrative systematic review.
Lasocki A, Abdalla G, Chow G, Thust SC
Cancer Imaging 2022 Nov 17;22(1):63. doi: 10.1186/s40644-022-00500-3. PMID: 36397143Free PMC Article

Recent systematic reviews

Imaging features associated with H3 K27-altered and H3 G34-mutant gliomas: a narrative systematic review.
Lasocki A, Abdalla G, Chow G, Thust SC
Cancer Imaging 2022 Nov 17;22(1):63. doi: 10.1186/s40644-022-00500-3. PMID: 36397143Free PMC Article

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