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Purpose <p>In the 2021 WHO classification system for central nervous system tumors, the diffuse glioma subgroup <i>IDH</i>-mutated (IDHm) astrocytomas WHO grade 4 was introduced. The diagnosis can be based upon molecular or histopathological morphological criteria. Here we explore whether phenotype and survival of IDHm astrocytomas WHO grade 4 differed across the criteria used for diagnosis.</p> Methods <p>Patients with IDHm astrocytoma, WHO grade 4, were included from Sahlgrenska University Hospital and TCGA database. We created three subgroups based upon the criteria for diagnosis of WHO grade 4; (1) homozygous <i>CDKN2A/B</i> deletion; (2) morphological (necrosis and/or microvascular proliferation); (3) combined subgroup with both homozygous <i>CDKN2A/B</i> deletion and morphological grade 4 criteria.</p> Results <p>We included 90 patients (local cohort, <i>n</i> = 35, TCGA cohort, <i>n</i> = 55) with IDHm astrocytoma, WHO grade 4. The median survival was 4.1 years (95% CI 3.0-5.3). Survival was comparable when the diagnosis was based on homozygous <i>CDKN2A/B</i> deletion and on morphological WHO grade 4 criteria (5.2 vs. 5.3 years). However, in the combined subgroup, survival was significantly shorter (2.8 years, <i>p</i> = 0.006).</p> Conclusion <p>The different subgroups of IDHm astrocytoma WHO grade 4 share similar characteristics. Patients whose tumors exhibit combined criteria have worse prognosis.</p>

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CDKN2A/B status versus morphology in diagnosing WHO grade 4 IDH-mutated astrocytomas: what is the clinical relevance?

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年5月20日

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  • Anna Lipatnikova,
  • Teresia Kling,
  • Anna Dénes,
  • Louise Carstam,
  • Alba Corell,
  • Malin Blomstrand,
  • Sandra Ferreyra Vega,
  • Dima Harba,
  • Thomas Olsson Bontell,
  • Helena Carén,
  • Asgeir S. Jakola

摘要

Purpose

In the 2021 WHO classification system for central nervous system tumors, the diffuse glioma subgroup IDH-mutated (IDHm) astrocytomas WHO grade 4 was introduced. The diagnosis can be based upon molecular or histopathological morphological criteria. Here we explore whether phenotype and survival of IDHm astrocytomas WHO grade 4 differed across the criteria used for diagnosis.

Methods

Patients with IDHm astrocytoma, WHO grade 4, were included from Sahlgrenska University Hospital and TCGA database. We created three subgroups based upon the criteria for diagnosis of WHO grade 4; (1) homozygous CDKN2A/B deletion; (2) morphological (necrosis and/or microvascular proliferation); (3) combined subgroup with both homozygous CDKN2A/B deletion and morphological grade 4 criteria.

Results

We included 90 patients (local cohort, n = 35, TCGA cohort, n = 55) with IDHm astrocytoma, WHO grade 4. The median survival was 4.1 years (95% CI 3.0-5.3). Survival was comparable when the diagnosis was based on homozygous CDKN2A/B deletion and on morphological WHO grade 4 criteria (5.2 vs. 5.3 years). However, in the combined subgroup, survival was significantly shorter (2.8 years, p = 0.006).

Conclusion

The different subgroups of IDHm astrocytoma WHO grade 4 share similar characteristics. Patients whose tumors exhibit combined criteria have worse prognosis.