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Recurrent Diffuse Midline Glioma, H3 K27M-Mutant

MedGen UID:
1791979
•Concept ID:
C5555538
•
Neoplastic Process

Definition

The reemergence of diffuse midline glioma, H3 K27M mutant, after a period of remission. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRecurrent Diffuse Midline Glioma, H3 K27M-Mutant

Recent clinical studies

Etiology

Pulvinar Locus is Highly Relevant to Patients' Outcomes in Surgically Resected Thalamic Gliomas in Children.
Chiba K, Aihara Y, Masui K, Abe K, Komori T, Kawamata T
World Neurosurg 2020 Feb;134:e530-e539. Epub 2019 Nov 5 doi: 10.1016/j.wneu.2019.10.116. PMID: 31704359
Pediatric and adult H3 K27M-mutant diffuse midline glioma treated with the selective DRD2 antagonist ONC201.
Chi AS, Tarapore RS, Hall MD, Shonka N, Gardner S, Umemura Y, Sumrall A, Khatib Z, Mueller S, Kline C, Zaky W, Khatua S, Weathers SP, Odia Y, Niazi TN, Daghistani D, Cherrick I, Korones D, Karajannis MA, Kong XT, Minturn J, Waanders A, Arillaga-Romany I, Batchelor T, Wen PY, Merdinger K, Schalop L, Stogniew M, Allen JE, Oster W, Mehta MP
J Neurooncol 2019 Oct;145(1):97-105. Epub 2019 Aug 27 doi: 10.1007/s11060-019-03271-3. PMID: 31456142Free PMC Article
Histone H3F3A and HIST1H3B K27M mutations define two subgroups of diffuse intrinsic pontine gliomas with different prognosis and phenotypes.
Castel D, Philippe C, Calmon R, Le Dret L, Truffaux N, Boddaert N, Pagès M, Taylor KR, Saulnier P, Lacroix L, Mackay A, Jones C, Sainte-Rose C, Blauwblomme T, Andreiuolo F, Puget S, Grill J, Varlet P, Debily MA
Acta Neuropathol 2015 Dec;130(6):815-27. Epub 2015 Sep 23 doi: 10.1007/s00401-015-1478-0. PMID: 26399631Free PMC Article

Diagnosis

A Rare High-Grade Glioma with a Histone H3 K27M Mutation in the Hypothalamus of an Adult Patient.
He P, Chen W, Qiu XX, Xi YB, Guan H, Xia J
World Neurosurg 2019 Aug;128:527-531. Epub 2019 Apr 29 doi: 10.1016/j.wneu.2019.04.172. PMID: 31048046
Histone H3F3A and HIST1H3B K27M mutations define two subgroups of diffuse intrinsic pontine gliomas with different prognosis and phenotypes.
Castel D, Philippe C, Calmon R, Le Dret L, Truffaux N, Boddaert N, Pagès M, Taylor KR, Saulnier P, Lacroix L, Mackay A, Jones C, Sainte-Rose C, Blauwblomme T, Andreiuolo F, Puget S, Grill J, Varlet P, Debily MA
Acta Neuropathol 2015 Dec;130(6):815-27. Epub 2015 Sep 23 doi: 10.1007/s00401-015-1478-0. PMID: 26399631Free PMC Article

Therapy

ONC201 (Dordaviprone) in Recurrent H3 K27M-Mutant Diffuse Midline Glioma.
Arrillaga-Romany I, Gardner SL, Odia Y, Aguilera D, Allen JE, Batchelor T, Butowski N, Chen C, Cloughesy T, Cluster A, de Groot J, Dixit KS, Graber JJ, Haggiagi AM, Harrison RA, Kheradpour A, Kilburn LB, Kurz SC, Lu G, MacDonald TJ, Mehta M, Melemed AS, Nghiemphu PL, Ramage SC, Shonka N, Sumrall A, Tarapore RS, Taylor L, Umemura Y, Wen PY
J Clin Oncol 2024 May 1;42(13):1542-1552. Epub 2024 Feb 9 doi: 10.1200/JCO.23.01134. PMID: 38335473
Pediatric and adult H3 K27M-mutant diffuse midline glioma treated with the selective DRD2 antagonist ONC201.
Chi AS, Tarapore RS, Hall MD, Shonka N, Gardner S, Umemura Y, Sumrall A, Khatib Z, Mueller S, Kline C, Zaky W, Khatua S, Weathers SP, Odia Y, Niazi TN, Daghistani D, Cherrick I, Korones D, Karajannis MA, Kong XT, Minturn J, Waanders A, Arillaga-Romany I, Batchelor T, Wen PY, Merdinger K, Schalop L, Stogniew M, Allen JE, Oster W, Mehta MP
J Neurooncol 2019 Oct;145(1):97-105. Epub 2019 Aug 27 doi: 10.1007/s11060-019-03271-3. PMID: 31456142Free PMC Article

Prognosis

ONC201 (Dordaviprone) in Recurrent H3 K27M-Mutant Diffuse Midline Glioma.
Arrillaga-Romany I, Gardner SL, Odia Y, Aguilera D, Allen JE, Batchelor T, Butowski N, Chen C, Cloughesy T, Cluster A, de Groot J, Dixit KS, Graber JJ, Haggiagi AM, Harrison RA, Kheradpour A, Kilburn LB, Kurz SC, Lu G, MacDonald TJ, Mehta M, Melemed AS, Nghiemphu PL, Ramage SC, Shonka N, Sumrall A, Tarapore RS, Taylor L, Umemura Y, Wen PY
J Clin Oncol 2024 May 1;42(13):1542-1552. Epub 2024 Feb 9 doi: 10.1200/JCO.23.01134. PMID: 38335473
Genomic and Phenotypic Characterization of a Broad Panel of Patient-Derived Xenografts Reflects the Diversity of Glioblastoma.
Vaubel RA, Tian S, Remonde D, Schroeder MA, Mladek AC, Kitange GJ, Caron A, Kollmeyer TM, Grove R, Peng S, Carlson BL, Ma DJ, Sarkar G, Evers L, Decker PA, Yan H, Dhruv HD, Berens ME, Wang Q, Marin BM, Klee EW, Califano A, LaChance DH, Eckel-Passow JE, Verhaak RG, Sulman EP, Burns TC, Meyer FB, O'Neill BP, Tran NL, Giannini C, Jenkins RB, Parney IF, Sarkaria JN
Clin Cancer Res 2020 Mar 1;26(5):1094-1104. Epub 2019 Dec 18 doi: 10.1158/1078-0432.CCR-19-0909. PMID: 31852831Free PMC Article
Pulvinar Locus is Highly Relevant to Patients' Outcomes in Surgically Resected Thalamic Gliomas in Children.
Chiba K, Aihara Y, Masui K, Abe K, Komori T, Kawamata T
World Neurosurg 2020 Feb;134:e530-e539. Epub 2019 Nov 5 doi: 10.1016/j.wneu.2019.10.116. PMID: 31704359
Pediatric and adult H3 K27M-mutant diffuse midline glioma treated with the selective DRD2 antagonist ONC201.
Chi AS, Tarapore RS, Hall MD, Shonka N, Gardner S, Umemura Y, Sumrall A, Khatib Z, Mueller S, Kline C, Zaky W, Khatua S, Weathers SP, Odia Y, Niazi TN, Daghistani D, Cherrick I, Korones D, Karajannis MA, Kong XT, Minturn J, Waanders A, Arillaga-Romany I, Batchelor T, Wen PY, Merdinger K, Schalop L, Stogniew M, Allen JE, Oster W, Mehta MP
J Neurooncol 2019 Oct;145(1):97-105. Epub 2019 Aug 27 doi: 10.1007/s11060-019-03271-3. PMID: 31456142Free PMC Article
Histone H3F3A and HIST1H3B K27M mutations define two subgroups of diffuse intrinsic pontine gliomas with different prognosis and phenotypes.
Castel D, Philippe C, Calmon R, Le Dret L, Truffaux N, Boddaert N, Pagès M, Taylor KR, Saulnier P, Lacroix L, Mackay A, Jones C, Sainte-Rose C, Blauwblomme T, Andreiuolo F, Puget S, Grill J, Varlet P, Debily MA
Acta Neuropathol 2015 Dec;130(6):815-27. Epub 2015 Sep 23 doi: 10.1007/s00401-015-1478-0. PMID: 26399631Free PMC Article

Clinical prediction guides

ONC201 (Dordaviprone) in Recurrent H3 K27M-Mutant Diffuse Midline Glioma.
Arrillaga-Romany I, Gardner SL, Odia Y, Aguilera D, Allen JE, Batchelor T, Butowski N, Chen C, Cloughesy T, Cluster A, de Groot J, Dixit KS, Graber JJ, Haggiagi AM, Harrison RA, Kheradpour A, Kilburn LB, Kurz SC, Lu G, MacDonald TJ, Mehta M, Melemed AS, Nghiemphu PL, Ramage SC, Shonka N, Sumrall A, Tarapore RS, Taylor L, Umemura Y, Wen PY
J Clin Oncol 2024 May 1;42(13):1542-1552. Epub 2024 Feb 9 doi: 10.1200/JCO.23.01134. PMID: 38335473
Pulvinar Locus is Highly Relevant to Patients' Outcomes in Surgically Resected Thalamic Gliomas in Children.
Chiba K, Aihara Y, Masui K, Abe K, Komori T, Kawamata T
World Neurosurg 2020 Feb;134:e530-e539. Epub 2019 Nov 5 doi: 10.1016/j.wneu.2019.10.116. PMID: 31704359
A Rare High-Grade Glioma with a Histone H3 K27M Mutation in the Hypothalamus of an Adult Patient.
He P, Chen W, Qiu XX, Xi YB, Guan H, Xia J
World Neurosurg 2019 Aug;128:527-531. Epub 2019 Apr 29 doi: 10.1016/j.wneu.2019.04.172. PMID: 31048046
Histone H3F3A and HIST1H3B K27M mutations define two subgroups of diffuse intrinsic pontine gliomas with different prognosis and phenotypes.
Castel D, Philippe C, Calmon R, Le Dret L, Truffaux N, Boddaert N, Pagès M, Taylor KR, Saulnier P, Lacroix L, Mackay A, Jones C, Sainte-Rose C, Blauwblomme T, Andreiuolo F, Puget S, Grill J, Varlet P, Debily MA
Acta Neuropathol 2015 Dec;130(6):815-27. Epub 2015 Sep 23 doi: 10.1007/s00401-015-1478-0. PMID: 26399631Free PMC Article

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