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Astrocytoma, IDH-mutant, grade 4

MedGen UID:
1718223
•Concept ID:
C5238471
•
Neoplastic Process
Synonyms: Astrocytoma, IDH-Mutant, Grade 4; astrocytoma, IDH-mutant, grade 4; Astrocytoma, IDH-Mutant, Grade IV
 
Monarch Initiative: MONDO:0956981

Definition

IDH-mutant astrocytoma characterized by the presence of necrosis and/or microvascular proliferation or homozygous deletion of CDKN2A and/or CDKN2B genes. The term glioblastoma no longer applies to central nervous system WHO grade 4 IDH-mutant astrocytomas. (WHO 2021) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAstrocytoma, IDH-mutant, grade 4

Professional guidelines

PubMed

SEOM-GEINO clinical guidelines for high-grade gliomas of adulthood (2022).
Segura PP, Quintela NV, García MM, Del Barco Berrón S, Sarrió RG, Gómez JG, Castaño AG, Martín LMN, Rubio OG, Losada EP
Clin Transl Oncol 2023 Sep;25(9):2634-2646. Epub 2023 Aug 4 doi: 10.1007/s12094-023-03245-y. PMID: 37540408Free PMC Article
Outcome and characteristics of patients with adult grade 4 diffuse gliomas changing sites of treatment.
Forster MT, Hug M, Geissler M, Voss M, Weber K, Hoelter MC, Seifert V, Czabanka M, Steinbach JP
J Cancer Res Clin Oncol 2023 Jan;149(1):111-119. Epub 2022 Nov 8 doi: 10.1007/s00432-022-04439-7. PMID: 36348019Free PMC Article
Combination Olaparib and Temozolomide for the Treatment of Glioma: A Retrospective Case Series.
Schaff LR, Kushnirsky M, Lin AL, Nandakumar S, Grommes C, Miller AM, Gavrilovic IT, Nolan C, Pentsova E, Mellinghoff IK, Kaley TJ
Neurology 2022 Oct 24;99(17):750-755. doi: 10.1212/WNL.0000000000201203. PMID: 35948444Free PMC Article

Recent clinical studies

Etiology

Clinical and molecular determinants of bleeding-related adverse outcomes in high-grade glioma.
Dasgupta P, Rousseau JF
J Neurooncol 2024 Feb;166(3):569-574. Epub 2024 Jan 29 doi: 10.1007/s11060-024-04574-w. PMID: 38286976
Supramaximal versus gross total resection in Glioblastoma, IDH wild-type and Astrocytoma, IDH-mutant, grade 4, effect on overall and progression free survival: systematic review and meta-analysis.
Mier-García JF, Ospina-Santa S, Orozco-Mera J, Ma R, Plaha P
J Neurooncol 2023 Aug;164(1):31-41. Epub 2023 Aug 10 doi: 10.1007/s11060-023-04409-0. PMID: 37561356

Diagnosis

Clinical and molecular determinants of bleeding-related adverse outcomes in high-grade glioma.
Dasgupta P, Rousseau JF
J Neurooncol 2024 Feb;166(3):569-574. Epub 2024 Jan 29 doi: 10.1007/s11060-024-04574-w. PMID: 38286976
Utility of real-time polymerase chain reaction for the assessment of CDKN2A homozygous deletion in adult-type IDH-mutant astrocytoma.
Shimizu Y, Suzuki M, Akiyama O, Ogino I, Matsushita Y, Satomi K, Yanagisawa S, Ohno M, Takahashi M, Miyakita Y, Narita Y, Ichimura K, Kondo A
Brain Tumor Pathol 2023 Apr;40(2):93-100. Epub 2023 Feb 14 doi: 10.1007/s10014-023-00450-z. PMID: 36788155

Therapy

Clinical and molecular determinants of bleeding-related adverse outcomes in high-grade glioma.
Dasgupta P, Rousseau JF
J Neurooncol 2024 Feb;166(3):569-574. Epub 2024 Jan 29 doi: 10.1007/s11060-024-04574-w. PMID: 38286976
Supramaximal versus gross total resection in Glioblastoma, IDH wild-type and Astrocytoma, IDH-mutant, grade 4, effect on overall and progression free survival: systematic review and meta-analysis.
Mier-García JF, Ospina-Santa S, Orozco-Mera J, Ma R, Plaha P
J Neurooncol 2023 Aug;164(1):31-41. Epub 2023 Aug 10 doi: 10.1007/s11060-023-04409-0. PMID: 37561356

Prognosis

Novel insight into histological and molecular astrocytoma, IDH-mutant, Grade 4 by the updated WHO classification of central nervous system tumors.
Chen W, Guo S, Wang Y, Shi Y, Guo X, Liu D, Li Y, Wang Y, Xing H, Xia Y, Li J, Wu J, Liang T, Wang H, Liu Q, Jin S, Qu T, Li H, Yang T, Zhang K, Wang Y, Ma W
Cancer Med 2023 Sep;12(18):18666-18678. Epub 2023 Sep 5 doi: 10.1002/cam4.6476. PMID: 37667984Free PMC Article
Vascular differences between IDH-wildtype glioblastoma and astrocytoma IDH-mutant grade 4 at imaging and transcriptomic levels.
Álvarez-Torres MDM, López-Cerdán A, Andreu Z, de la Iglesia Vayá M, Fuster-Garcia E, García-García F, García-Gómez JM
NMR Biomed 2023 Nov;36(11):e5004. Epub 2023 Jul 21 doi: 10.1002/nbm.5004. PMID: 37482922
Machine learning to improve interpretability of clinical, radiological and panel-based genomic data of glioma grade 4 patients undergoing surgical resection.
Dal Bo M, Polano M, Ius T, Di Cintio F, Mondello A, Manini I, Pegolo E, Cesselli D, Di Loreto C, Skrap M, Toffoli G
J Transl Med 2023 Jul 7;21(1):450. doi: 10.1186/s12967-023-04308-y. PMID: 37420248Free PMC Article
Utility of real-time polymerase chain reaction for the assessment of CDKN2A homozygous deletion in adult-type IDH-mutant astrocytoma.
Shimizu Y, Suzuki M, Akiyama O, Ogino I, Matsushita Y, Satomi K, Yanagisawa S, Ohno M, Takahashi M, Miyakita Y, Narita Y, Ichimura K, Kondo A
Brain Tumor Pathol 2023 Apr;40(2):93-100. Epub 2023 Feb 14 doi: 10.1007/s10014-023-00450-z. PMID: 36788155
A map of the spatial distribution and tumour-associated macrophage states in glioblastoma and grade 4 IDH-mutant astrocytoma.
Yin W, Ping YF, Li F, Lv SQ, Zhang XN, Li XG, Guo Y, Liu Q, Li TR, Yang LQ, Yang KD, Liu YQ, Luo CH, Luo T, Wang WY, Mao M, Luo M, He ZC, Cao MF, Chen C, Miao JY, Zeng H, Wang C, Zhou L, Yang Y, Yang X, Wang QH, Feng H, Shi Y, Bian XW
J Pathol 2022 Oct;258(2):121-135. Epub 2022 Jul 25 doi: 10.1002/path.5984. PMID: 35723032

Clinical prediction guides

Novel insight into histological and molecular astrocytoma, IDH-mutant, Grade 4 by the updated WHO classification of central nervous system tumors.
Chen W, Guo S, Wang Y, Shi Y, Guo X, Liu D, Li Y, Wang Y, Xing H, Xia Y, Li J, Wu J, Liang T, Wang H, Liu Q, Jin S, Qu T, Li H, Yang T, Zhang K, Wang Y, Ma W
Cancer Med 2023 Sep;12(18):18666-18678. Epub 2023 Sep 5 doi: 10.1002/cam4.6476. PMID: 37667984Free PMC Article
Vascular differences between IDH-wildtype glioblastoma and astrocytoma IDH-mutant grade 4 at imaging and transcriptomic levels.
Álvarez-Torres MDM, López-Cerdán A, Andreu Z, de la Iglesia Vayá M, Fuster-Garcia E, García-García F, García-Gómez JM
NMR Biomed 2023 Nov;36(11):e5004. Epub 2023 Jul 21 doi: 10.1002/nbm.5004. PMID: 37482922
Machine learning to improve interpretability of clinical, radiological and panel-based genomic data of glioma grade 4 patients undergoing surgical resection.
Dal Bo M, Polano M, Ius T, Di Cintio F, Mondello A, Manini I, Pegolo E, Cesselli D, Di Loreto C, Skrap M, Toffoli G
J Transl Med 2023 Jul 7;21(1):450. doi: 10.1186/s12967-023-04308-y. PMID: 37420248Free PMC Article

Recent systematic reviews

Supramaximal versus gross total resection in Glioblastoma, IDH wild-type and Astrocytoma, IDH-mutant, grade 4, effect on overall and progression free survival: systematic review and meta-analysis.
Mier-García JF, Ospina-Santa S, Orozco-Mera J, Ma R, Plaha P
J Neurooncol 2023 Aug;164(1):31-41. Epub 2023 Aug 10 doi: 10.1007/s11060-023-04409-0. PMID: 37561356

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