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High-Grade Astrocytoma With Piloid Features: Case Series and Review of a Recently Described Brain Tumor Entity.

Mark A Rudolf1, Sean P Ferris1

  • 1From the Department of Pathology, University of Michigan Medical School, Ann Arbor.

Archives of Pathology & Laboratory Medicine
|January 23, 2025
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Summary

High-grade astrocytoma with piloid features (HGAP) is a newly recognized glioma. Recent studies refine understanding of its molecular features, subtypes, and associations, aiding diagnosis through methylation analysis.

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Area of Science:

  • Neuro-oncology
  • Molecular Pathology
  • Genetics

Background:

  • High-grade astrocytoma with piloid features (HGAP) is a recently defined glioma characterized by its distinct methylation profile.
  • Understanding its clinical, histological, and molecular features is crucial for accurate diagnosis and management.

Purpose of the Study:

  • To review the latest literature on HGAP, focusing on advancements since its 2021 World Health Organization (WHO) classification.
  • To present a case series from a single institution detailing their experience with HGAP.

Main Methods:

  • Comprehensive review of English-language HGAP literature published between 2018 and 2024.
  • Retrospective analysis of four HGAP cases, including review of associated medical records.

Main Results:

  • HGAP is increasingly recognized in adults and is a key differential diagnosis for isocitrate dehydrogenase-wild-type gliomas.
  • Updated understanding includes three provisional subtypes, association with neurofibromatosis 1 syndrome, rare molecular alterations, and a potential pilocytic astrocytoma to HGAP transformation.
  • Diagnostic clues include specific histological and molecular findings (e.g., CDKN2A/B deletion, MAPK pathway alterations, ATRX loss), necessitating molecular testing.

Conclusions:

  • HGAP diagnosis relies heavily on genome-wide DNA methylation analysis.
  • Recognizing HGAP's evolving characteristics and diagnostic indicators is vital for neuropathologists and neuro-oncologists.
  • Further research will continue to refine the understanding and classification of this glioma subtype.