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Diffuse astrocytic glioma, IDH-Wildtype, with molecular features of glioblastoma, WHO grade IV: A single-institution case series and review

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Journal of Neuro-Oncology Aims and scope Submit manuscript

Abstract

Objective

In 2018, cIMPACT-NOW update 3 concluded that WHO grade II/III IDH-wildtype diffuse astrocytomas that contain TERT promoter mutations, chromosome 7 gain/10 loss, and/or EGFR amplification, correspond to a WHO grade IV diagnosis and should be classified as Diffuse astrocytic glioma, IDH-wildtype, with molecular features of glioblastoma, WHO grade IV (DAG-G). We present a single-institution series of patients with DAG-G and IDH-mutant astrocytomas and compare their clinical, molecular, and radiographic characteristics.

Methods

Patient data was retrospectively extracted from the EMR for all patients undergoing surgical biopsy/resection of a diffuse astrocytoma at our institution from 2018 to 2020. Clinical presentation, molecular alterations, radiographic appearance, surgery, and survival were reviewed for each patient.

Results

Six DAG-G patients were identified in our cohort. All patients had diffuse disease, and presented with expansile, T2 hyperintense lesions with minimal enhancement. Compared to patients with classic IDH-mutant astrocytomas, mean age for DAG-G patients was older (68 vs 33 years, p < 0.0001), tumors were more diffuse (p = 0.02), with patients more likely to present with focal deficits and receive a biopsy only (p = 0.005). Overall survival was significantly shorter for DAG-G patients (p = 0.03).

Conclusion

Patients with DAG-G are more likely to be older than typical IDH-mutant diffuse astrocytoma patients. They are more likely to present with tumors in a diffuse pattern with focal deficits. When such patients are encountered, prompt biopsy/resection to confirm the diagnosis and immediate initiation of adjuvant therapy is recommended, as the disease progression and overall prognosis is similar to glioblastoma.

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Data availability

Data and material available by request

Code availability

Not applicable

Abbreviations

ATRX:

Alpha-thalassemia/mental retardation syndrome X-linked

cIMPACT-NOW:

The consortium to inform molecular and practical approaches to CNS tumor taxonomy

CNS:

Central nervous system

DAG-G:

Diffuse astrocytic glioma, IDH-wildtype, with molecular features of glioblastoma, WHO grade IV

EGFR:

Epidermal growth factor receptor

FLAIR:

Fluid attenuation inversion recovery

GBM:

Glioblastoma

GTR:

Gross-Total resection

IDH:

Isocitrate dehydrogenase

MGMT:

O-6-Methylguanine-DNA methyltransferase

MRI:

Magnetic resonance imaging

OS:

Overall survival

PFS:

Progression-free survival

PTEN:

Phosphatase and Tensin homolog

STR:

Sub-Total resection

TERT:

Telomerase reverse transcriptase

WHO:

World Health Organization

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Authors and Affiliations

  1. Department of Neurological Surgery, University of California Davis, Sacramento, CA, USA

    Dennis Lee, Robert A. Riestenberg, Aden Haskell-Mendoza & Orin Bloch

  2. Chicago Medical School, Rosalind Franklin University of Medicine and Science, North Chicago, IL, USA

    Dennis Lee

  3. Northwestern University, Feinberg School of Medicine, Chicago, IL, USA

    Robert A. Riestenberg

  4. Duke University School of Medicine, Durham, NC, USA

    Aden Haskell-Mendoza

Authors
  1. Dennis Lee
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  2. Robert A. Riestenberg
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  3. Aden Haskell-Mendoza
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  4. Orin Bloch
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Correspondence to Orin Bloch.

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Lee, D., Riestenberg, R.A., Haskell-Mendoza, A. et al. Diffuse astrocytic glioma, IDH-Wildtype, with molecular features of glioblastoma, WHO grade IV: A single-institution case series and review. J Neurooncol 152, 89–98 (2021). https://doi.org/10.1007/s11060-020-03677-4

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  • DOI: https://doi.org/10.1007/s11060-020-03677-4

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