Pathology · CNS Pathology (Tumors, Degenerative, Infections)

According to the 2021 WHO Classification of CNS Tumors, a diffuse glioma in a 30-year-old with IDH1 R132H mutation and 1p/19q codeletion is classified as:

  • A Astrocytoma, IDH-mutant (grade 2 or 3)
  • B Glioblastoma, IDH-mutant
  • C Oligodendroglioma, IDH-mutant and 1p/19q-codeleted (WHO grade 2 or 3)
  • D Diffuse midline glioma, H3 K27-altered
Correct answer: C. Oligodendroglioma, IDH-mutant and 1p/19q-codeleted (WHO grade 2 or 3)

Explanation

WHO 2021 CNS classification integrates molecular markers as diagnostic criteria. Diffuse gliomas with IDH mutation AND 1p/19q codeletion are definitionally oligodendrogliomas; the 1p/19q codeletion results from an unbalanced translocation and is accompanied by CIC and FUBP1 mutations. IDH-mutant gliomas without 1p/19q codeletion (but with ATRX loss) are astrocytomas. IDH-wildtype diffuse glioma with EGFR amplification, TERT mutation, or +7/−10 is GBM. H3 K27-altered defines diffuse midline glioma.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

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