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Astrocytoma, IDH-mutant, grade 2

MedGen UID:
927224
•Concept ID:
C4289692
•
Neoplastic Process
Synonyms: Astrocytoma, IDH-Mutant, Grade 2; astrocytoma, IDH-mutant, grade 2; Astrocytoma, IDH-Mutant, Grade II; Diffuse Astrocytoma, IDH-Mutant
 
Monarch Initiative: MONDO:0956994

Definition

IDH-mutant astrocytoma characterized by the presence of well-differentiated fibrillary glial cells diffusely infiltrating the central nervous system. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAstrocytoma, IDH-mutant, grade 2

Professional guidelines

PubMed

Long-term treatment outcomes of temozolomide-based chemoradiation in patients with adult-type diffuse IDH-mutant grade 2 astrocytoma.
Minniti G, Paolini S, Antonelli M, Gianno F, Tini P, Lanzetta G, Arcella A, De Pietro R, Giraffa M, Capone L, Romano A, Bozzao A, Esposito V
J Neurooncol 2023 Sep;164(2):331-339. Epub 2023 Sep 4 doi: 10.1007/s11060-023-04418-z. PMID: 37665475Free 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

Interactive Effects of Molecular, Therapeutic, and Patient Factors on Outcome of Diffuse Low-Grade Glioma.
Hervey-Jumper SL, Zhang Y, Phillips JJ, Morshed RA, Young JS, McCoy L, Lafontaine M, Luks T, Ammanuel S, Kakaizada S, Egladyous A, Gogos A, Villanueva-Meyer J, Shai A, Warrier G, Rice T, Crane J, Wrensch M, Wiencke JK, Daras M, Oberheim Bush NA, Taylor JW, Butowski N, Clarke J, Chang S, Chang E, Aghi M, Theodosopoulos P, McDermott M, Jakola AS, Kavouridis VK, Nawabi N, Solheim O, Smith T, Berger MS, Molinaro AM
J Clin Oncol 2023 Apr 10;41(11):2029-2042. Epub 2023 Jan 4 doi: 10.1200/JCO.21.02929. PMID: 36599113Free PMC Article
Grading of IDH-mutant astrocytoma using diffusion, susceptibility and perfusion-weighted imaging.
Yang X, Xing Z, She D, Lin Y, Zhang H, Su Y, Cao D
BMC Med Imaging 2022 May 29;22(1):105. doi: 10.1186/s12880-022-00832-3. PMID: 35644621Free PMC Article
Grading of adult diffuse gliomas according to the 2021 WHO Classification of Tumors of the Central Nervous System.
Komori T
Lab Invest 2022 Feb;102(2):126-133. Epub 2021 Sep 9 doi: 10.1038/s41374-021-00667-6. PMID: 34504304
CDKN2A homozygous deletion is a strong adverse prognosis factor in diffuse malignant IDH-mutant gliomas.
Appay R, Dehais C, Maurage CA, Alentorn A, Carpentier C, Colin C, Ducray F, Escande F, Idbaih A, Kamoun A, Marie Y, Mokhtari K, Tabouret E, Trabelsi N, Uro-Coste E, Delattre JY, Figarella-Branger D; POLA Network
Neuro Oncol 2019 Dec 17;21(12):1519-1528. doi: 10.1093/neuonc/noz124. PMID: 31832685Free PMC Article
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742

Diagnosis

NCCN Guidelines® Insights: Central Nervous System Cancers, Version 2.2022.
Horbinski C, Nabors LB, Portnow J, Baehring J, Bhatia A, Bloch O, Brem S, Butowski N, Cannon DM, Chao S, Chheda MG, Fabiano AJ, Forsyth P, Gigilio P, Hattangadi-Gluth J, Holdhoff M, Junck L, Kaley T, Merrell R, Mrugala MM, Nagpal S, Nedzi LA, Nevel K, Nghiemphu PL, Parney I, Patel TR, Peters K, Puduvalli VK, Rockhill J, Rusthoven C, Shonka N, Swinnen LJ, Weiss S, Wen PY, Willmarth NE, Bergman MA, Darlow S
J Natl Compr Canc Netw 2023 Jan;21(1):12-20. doi: 10.6004/jnccn.2023.0002. PMID: 36634606
Translational significance of CDKN2A/B homozygous deletion in isocitrate dehydrogenase-mutant astrocytoma.
Fortin Ensign SP, Jenkins RB, Giannini C, Sarkaria JN, Galanis E, Kizilbash SH
Neuro Oncol 2023 Jan 5;25(1):28-36. doi: 10.1093/neuonc/noac205. PMID: 35973817Free PMC Article
Therapy for Diffuse Astrocytic and Oligodendroglial Tumors in Adults: ASCO-SNO Guideline.
Mohile NA, Messersmith H, Gatson NT, Hottinger AF, Lassman A, Morton J, Ney D, Nghiemphu PL, Olar A, Olson J, Perry J, Portnow J, Schiff D, Shannon A, Shih HA, Strowd R, van den Bent M, Ziu M, Blakeley J
J Clin Oncol 2022 Feb 1;40(4):403-426. Epub 2021 Dec 13 doi: 10.1200/JCO.21.02036. PMID: 34898238
Grading of adult diffuse gliomas according to the 2021 WHO Classification of Tumors of the Central Nervous System.
Komori T
Lab Invest 2022 Feb;102(2):126-133. Epub 2021 Sep 9 doi: 10.1038/s41374-021-00667-6. PMID: 34504304
IDH-Mutant Low-grade Glioma: Advances in Molecular Diagnosis, Management, and Future Directions.
Dono A, Ballester LY, Primdahl D, Esquenazi Y, Bhatia A
Curr Oncol Rep 2021 Jan 25;23(2):20. doi: 10.1007/s11912-020-01006-6. PMID: 33492489

Therapy

Vorasidenib: First Approval.
Lamb YN
Drugs 2024 Oct;84(10):1325-1331. Epub 2024 Oct 8 doi: 10.1007/s40265-024-02097-2. PMID: 39375303
IDH inhibition in gliomas: from preclinical models to clinical trials.
Rudà R, Horbinski C, van den Bent M, Preusser M, Soffietti R
Nat Rev Neurol 2024 Jul;20(7):395-407. Epub 2024 May 17 doi: 10.1038/s41582-024-00967-7. PMID: 38760442
Interactive Effects of Molecular, Therapeutic, and Patient Factors on Outcome of Diffuse Low-Grade Glioma.
Hervey-Jumper SL, Zhang Y, Phillips JJ, Morshed RA, Young JS, McCoy L, Lafontaine M, Luks T, Ammanuel S, Kakaizada S, Egladyous A, Gogos A, Villanueva-Meyer J, Shai A, Warrier G, Rice T, Crane J, Wrensch M, Wiencke JK, Daras M, Oberheim Bush NA, Taylor JW, Butowski N, Clarke J, Chang S, Chang E, Aghi M, Theodosopoulos P, McDermott M, Jakola AS, Kavouridis VK, Nawabi N, Solheim O, Smith T, Berger MS, Molinaro AM
J Clin Oncol 2023 Apr 10;41(11):2029-2042. Epub 2023 Jan 4 doi: 10.1200/JCO.21.02929. PMID: 36599113Free PMC Article
Therapy for Diffuse Astrocytic and Oligodendroglial Tumors in Adults: ASCO-SNO Guideline.
Mohile NA, Messersmith H, Gatson NT, Hottinger AF, Lassman A, Morton J, Ney D, Nghiemphu PL, Olar A, Olson J, Perry J, Portnow J, Schiff D, Shannon A, Shih HA, Strowd R, van den Bent M, Ziu M, Blakeley J
J Clin Oncol 2022 Feb 1;40(4):403-426. Epub 2021 Dec 13 doi: 10.1200/JCO.21.02036. PMID: 34898238
Glutamate and α-ketoglutarate: key players in glioma metabolism.
Maus A, Peters GJ
Amino Acids 2017 Jan;49(1):21-32. Epub 2016 Oct 17 doi: 10.1007/s00726-016-2342-9. PMID: 27752843Free PMC Article

Prognosis

Translational significance of CDKN2A/B homozygous deletion in isocitrate dehydrogenase-mutant astrocytoma.
Fortin Ensign SP, Jenkins RB, Giannini C, Sarkaria JN, Galanis E, Kizilbash SH
Neuro Oncol 2023 Jan 5;25(1):28-36. doi: 10.1093/neuonc/noac205. PMID: 35973817Free PMC Article
Therapy for Diffuse Astrocytic and Oligodendroglial Tumors in Adults: ASCO-SNO Guideline.
Mohile NA, Messersmith H, Gatson NT, Hottinger AF, Lassman A, Morton J, Ney D, Nghiemphu PL, Olar A, Olson J, Perry J, Portnow J, Schiff D, Shannon A, Shih HA, Strowd R, van den Bent M, Ziu M, Blakeley J
J Clin Oncol 2022 Feb 1;40(4):403-426. Epub 2021 Dec 13 doi: 10.1200/JCO.21.02036. PMID: 34898238
Grading of adult diffuse gliomas according to the 2021 WHO Classification of Tumors of the Central Nervous System.
Komori T
Lab Invest 2022 Feb;102(2):126-133. Epub 2021 Sep 9 doi: 10.1038/s41374-021-00667-6. PMID: 34504304
CDKN2A homozygous deletion is a strong adverse prognosis factor in diffuse malignant IDH-mutant gliomas.
Appay R, Dehais C, Maurage CA, Alentorn A, Carpentier C, Colin C, Ducray F, Escande F, Idbaih A, Kamoun A, Marie Y, Mokhtari K, Tabouret E, Trabelsi N, Uro-Coste E, Delattre JY, Figarella-Branger D; POLA Network
Neuro Oncol 2019 Dec 17;21(12):1519-1528. doi: 10.1093/neuonc/noz124. PMID: 31832685Free PMC Article
Glioblastoma: pathology, molecular mechanisms and markers.
Aldape K, Zadeh G, Mansouri S, Reifenberger G, von Deimling A
Acta Neuropathol 2015 Jun;129(6):829-48. Epub 2015 May 6 doi: 10.1007/s00401-015-1432-1. PMID: 25943888

Clinical prediction guides

Interactive Effects of Molecular, Therapeutic, and Patient Factors on Outcome of Diffuse Low-Grade Glioma.
Hervey-Jumper SL, Zhang Y, Phillips JJ, Morshed RA, Young JS, McCoy L, Lafontaine M, Luks T, Ammanuel S, Kakaizada S, Egladyous A, Gogos A, Villanueva-Meyer J, Shai A, Warrier G, Rice T, Crane J, Wrensch M, Wiencke JK, Daras M, Oberheim Bush NA, Taylor JW, Butowski N, Clarke J, Chang S, Chang E, Aghi M, Theodosopoulos P, McDermott M, Jakola AS, Kavouridis VK, Nawabi N, Solheim O, Smith T, Berger MS, Molinaro AM
J Clin Oncol 2023 Apr 10;41(11):2029-2042. Epub 2023 Jan 4 doi: 10.1200/JCO.21.02929. PMID: 36599113Free PMC Article
Therapy for Diffuse Astrocytic and Oligodendroglial Tumors in Adults: ASCO-SNO Guideline.
Mohile NA, Messersmith H, Gatson NT, Hottinger AF, Lassman A, Morton J, Ney D, Nghiemphu PL, Olar A, Olson J, Perry J, Portnow J, Schiff D, Shannon A, Shih HA, Strowd R, van den Bent M, Ziu M, Blakeley J
J Clin Oncol 2022 Feb 1;40(4):403-426. Epub 2021 Dec 13 doi: 10.1200/JCO.21.02036. PMID: 34898238
Grading of adult diffuse gliomas according to the 2021 WHO Classification of Tumors of the Central Nervous System.
Komori T
Lab Invest 2022 Feb;102(2):126-133. Epub 2021 Sep 9 doi: 10.1038/s41374-021-00667-6. PMID: 34504304
Molecular landscape of IDH-mutant primary astrocytoma Grade IV/glioblastomas.
Wong QH, Li KK, Wang WW, Malta TM, Noushmehr H, Grabovska Y, Jones C, Chan AK, Kwan JS, Huang QJ, Wong GC, Li WC, Liu XZ, Chen H, Chan DT, Mao Y, Zhang ZY, Shi ZF, Ng HK
Mod Pathol 2021 Jul;34(7):1245-1260. Epub 2021 Mar 10 doi: 10.1038/s41379-021-00778-x. PMID: 33692446
Glioblastoma: pathology, molecular mechanisms and markers.
Aldape K, Zadeh G, Mansouri S, Reifenberger G, von Deimling A
Acta Neuropathol 2015 Jun;129(6):829-48. Epub 2015 May 6 doi: 10.1007/s00401-015-1432-1. PMID: 25943888

Recent systematic reviews

Molecular markers related to patient outcome in patients with IDH-mutant astrocytomas grade 2 to 4: A systematic review.
Tesileanu CMS, Vallentgoed WR, French PJ, van den Bent MJ
Eur J Cancer 2022 Nov;175:214-223. Epub 2022 Sep 21 doi: 10.1016/j.ejca.2022.08.016. PMID: 36152406
Radiation Therapy for IDH-Mutant Grade 2 and Grade 3 Diffuse Glioma: An ASTRO Clinical Practice Guideline.
Halasz LM, Attia A, Bradfield L, Brat DJ, Kirkpatrick JP, Laack NN, Lalani N, Lebow ES, Liu AK, Niemeier HM, Palmer JD, Peters KB, Sheehan J, Thomas RP, Vora SA, Wahl DR, Weiss SE, Yeboa DN, Zhong J, Shih HA
Pract Radiat Oncol 2022 Sep-Oct;12(5):370-386. Epub 2022 May 31 doi: 10.1016/j.prro.2022.05.004. PMID: 35902341
Therapy for Diffuse Astrocytic and Oligodendroglial Tumors in Adults: ASCO-SNO Guideline.
Mohile NA, Messersmith H, Gatson NT, Hottinger AF, Lassman A, Morton J, Ney D, Nghiemphu PL, Olar A, Olson J, Perry J, Portnow J, Schiff D, Shannon A, Shih HA, Strowd R, van den Bent M, Ziu M, Blakeley J
J Clin Oncol 2022 Feb 1;40(4):403-426. Epub 2021 Dec 13 doi: 10.1200/JCO.21.02036. PMID: 34898238
The role of 2-hydroxyglutarate magnetic resonance spectroscopy for the determination of isocitrate dehydrogenase status in lower grade gliomas versus glioblastoma: a systematic review and meta-analysis of diagnostic test accuracy.
Bhandari A, Sharma C, Ibrahim M, Riggs M, Jones R, Lasocki A
Neuroradiology 2021 Nov;63(11):1823-1830. Epub 2021 Apr 3 doi: 10.1007/s00234-021-02702-1. PMID: 33811494
The T2-FLAIR mismatch sign as a predictor of IDH-mutant, 1p/19q-noncodeleted lower-grade gliomas: a systematic review and diagnostic meta-analysis.
Park SI, Suh CH, Guenette JP, Huang RY, Kim HS
Eur Radiol 2021 Jul;31(7):5289-5299. Epub 2021 Jan 6 doi: 10.1007/s00330-020-07467-4. PMID: 33409784

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