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Review
. 2022 Sep;23(9):1219-1232.
doi: 10.1007/s11864-022-01000-z. Epub 2022 Aug 1.

Current Considerations in the Treatment of Grade 3 Gliomas

Jasmin Jo  1 , David Schiff  2
Affiliations
Review

Current Considerations in the Treatment of Grade 3 Gliomas

Jasmin Jo et al. Curr Treat Options Oncol. 2022 Sep.

Abstract

Treatment recommendations for grade 3 gliomas are guided by their histopathologic and molecular phenotype. In the 2021 WHO classification, these tumors are categorized into two types, grade 3 IDH mutant (IDHmt), 1p/19q codeleted oligodendroglioma and IDH mutant astrocytoma. Treatment consists of maximal safe surgery, followed by radiation therapy (RT) and alkylating agent-based chemotherapy. Based on the updated CATNON result, RT followed by temozolomide improves outcome in patients with non-codeleted grade 3 IDHmt astrocytoma. In patients with IDHmt, codeleted oligodendroglioma, the addition of procarbazine, CCNU, and vincristine regimen is the recommended treatment, based on large randomized controlled trials. These current treatments prolong the overall survival to up to 10 years in patients with grade 3 IDHmt astrocytoma and 14 years in grade 3 IDHmt codeleted oligodendroglioma. Treatment options at recurrence include re-resection, re-irradiation, and other cytotoxic chemotherapy; however, these are of limited benefit. Novel agents targeting IDH mutation and its metabolic effects are currently under investigation to improve the outcome of these patients.

Keywords: 1p/19q codeletion; IDH inhibitors; IDH mutation; Oligodendroglioma; WHO grade 3 astrocytoma.

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References

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
    1. • Yan H, Parsons DW, Jin G, McLendon R, Rasheed BA, Yuan W, et al. IDH1 and IDH2 mutations in gliomas. N Engl J Med. 2009;360(8):765-73. This paper first described the high frequency of IDH mutation in gliomas.
    1. Balss J, Meyer J, Mueller W, Korshunov A, Hartmann C, von Deimling A. Analysis of the IDH1 codon 132 mutation in brain tumors. Acta Neuropathol. 2008;116(6):597–602. - DOI
    1. Olar A, Wani KM, Diefes K, Heathcock LE, van Thuijl HF, Gilbert MR, et al. IDH mutation status and role of WHO grade and mitotic index in overall survival in grade II-III diffuse gliomas. Acta Neuropathol. 2015;129(4):585–96. - DOI
    1. •• Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021;23(8):1231-51. This paper discusses the new classification and grading of CNS tumors based on the molecular phenotypes of the tumors.
    1. •• Lassman A, Won M, Cairncross JG, Shaw EG, Ashby L, Souhami L, et al. ACTR-13. Final results with chemoradiotherapy for anaplastic oligodendroglial tumors from NRG oncology/RTOG 9402. Neuro-Oncology. 2019;21(6):vi15. This is one of the major RCT that demonstrates survival benefit with the addition of PCV to radiation therapy in grade 3 oligodendroglioma.

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