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  • Updated 01.03.2024
  • Released 01.16.2020
  • Expires For CME 01.03.2027

Glioblastoma, IDH wildtype



Glioblastoma is the most common primary malignant brain tumor in the adult population. The 2021 World Health Organization (WHO) Classification of Tumors of the Central Nervous System revised adult-type diffuse gliomas into three distinct tumor types: (1) glioblastoma, isocitrate dehydrogenase (IDH) wildtype, CNS WHO grade 4; (2) astrocytoma, IDH-mutant, CNS WHO grade 2-4; (3) oligodendroglioma, IDH-mutant, 1p/19q-codeleted, CNS WHO grade 2-3. This article focuses specifically on the diagnosis and management of glioblastoma, IDH wildtype, CNS WHO grade 4.

Key points

• Glioblastoma, IDH wildtype, is the most common malignant primary brain tumor with an often-aggressive clinical course.

• Treatment of glioblastoma, IDH wildtype, is multimodal and includes maximal safe surgical resection, fractionated radiation, chemotherapy, and tumor-treating fields.

• Age and performance status at diagnosis in glioblastoma IDH wildtype may influence optimal dosing of radiation and chemotherapy.

• Molecular testing should be considered in glial neoplasms that are IDH1 R132H wildtype by immunohistochemistry with high clinical or radiologic suspicion for other high-grade gliomas, including non-canonical IDH-mutated high-grade gliomas, diffuse midline gliomas with H3 K27-altered, and diffuse hemispheric gliomas with H3 G34 mutated; potential targetable mutations should be evaluated.

• Astrocytomas, IDH mutant WHO grade 4; diffuse midline gliomas with H3 K27-altered; diffuse hemispheric gliomas with H3 G34 mutation; pleomorphic xanthoastrocytomas; and other distinct entities are defined by other features and are not discussed in detail in this review.

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ISSN: 2831-9125