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IDH-Mutant Low-grade Glioma: Advances in Molecular Diagnosis, Management, and Future Directions

  • Neuro-oncology (KS Nevel, Section Editor)
  • Published:
Current Oncology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

IDH-mutant low-grade gliomas (LGG) have emerged as a distinct clinical and molecular entity with unique treatment considerations. Here, we review updates in IDH-mutant LGG diagnosis and classification, imaging biomarkers, therapies, and neurocognitive and patient-reported outcomes.

Recent Findings

CDKN2A/B homozygous deletion in IDH-mutant astrocytoma is associated with shorter survival, similar to WHO grade 4. The T2-FLAIR mismatch, a highly specific but insensitive sign, is diagnostic of IDH-mutant astrocytoma. Maximal safe resection is currently indicated in all LGG cases. Radiotherapy with subsequent PCV (procarbazine, lomustine, vincristine) provides longer overall survival compared to radiotherapy alone. Temozolomide in place of PCV is reasonable, but high-level evidence is still lacking. LGG adjuvant treatment has important quality of life and neurocognitive side effects that should be considered.

Summary

Although incurable, IDH-mutant LGG have a favorable survival compared to IDH-WT glioma. Recent advances in molecular-based classification, imaging, and targeted therapies will hopefully improve survival and quality of life.

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Data Availability

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Authors and Affiliations

  1. Vivian L. Smith Department of Neurosurgery, The University of Texas Health Science Center, 6431 Fannin Street, MSB 3.000, Houston, TX, 77030, USA

    Antonio Dono, Leomar Y. Ballester & Yoshua Esquenazi

  2. Department of Pathology and Laboratory Medicine, The University of Texas Health Science Center, 6431 Fannin St., MSB 2.136, Houston, TX, 77030, USA

    Antonio Dono & Leomar Y. Ballester

  3. Memorial Hermann Health System, Houston, TX, USA

    Leomar Y. Ballester, Yoshua Esquenazi & Ankush Bhatia

  4. Department of Neurology, University of Wisconsin School of Medicine and Public Health, 600 Highland Ave, Madison, WI, 53792, USA

    Ditte Primdahl

  5. Center for Precision Health, School of Biomedical Informatics, The University of Texas Health Science Center, 6400 Fannin Street, Suite # 2800, Houston, TX, 77030, USA

    Yoshua Esquenazi

  6. Department of Neurology, The University of Texas Health Science Center at Houston – McGovern Medical School, 6410 Fannin Street, Suite # 1014, Houston, TX, 77030, USA

    Ankush Bhatia

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Dono, A., Ballester, L.Y., Primdahl, D. et al. IDH-Mutant Low-grade Glioma: Advances in Molecular Diagnosis, Management, and Future Directions. Curr Oncol Rep 23, 20 (2021). https://doi.org/10.1007/s11912-020-01006-6

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