Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
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Questions and Answers

What is the most common location of oligodendroglioma, IDH-mutant and 1p/19q-codeleted, based on the CBTRUS database?

  • Parietal lobe
  • Frontal lobe (correct)
  • Temporal lobe
  • Occipital lobe

    • What is the WHO grade of oligodendroglioma, IDH-mutant and 1p/19q-codeleted?

  • Grade 2 or 3 (correct)
  • Grade 1
  • Not graded
  • Grade 4

    • What is the ICD-O coding for oligodendroglioma, IDH-mutant and 1p/19q-codeleted, grade 3?

  • 9453/3
  • 9452/3
  • 9451/3 (correct)
  • 9450/3

    • What percentage of genetically defined CNS WHO grade 3 oligodendrogliomas were located in the frontal lobe in the French national POLA network?

    <p>62%</p> Signup and view all the answers

    What is not recommended in the terminology of oligodendroglioma, IDH-mutant and 1p/19q-codeleted?

    <p>Anaplastic oligodendroglioma, IDH-mutant and 1p/19q-codeleted</p> Signup and view all the answers

    What is the ICD-11 coding for oligodendroglioma, IDH-mutant and 1p/19q-codeleted?

    <p>2A00.0Y &amp; XH7K31</p> Signup and view all the answers

    What is the most common IDH mutation found in oligodendrogliomas?

    <p>p.R132H</p> Signup and view all the answers

    What is the result of an unbalanced translocation between chromosomes 1 and 19?

    <p>Retention of the der(1;19)(q10;p10) chromosome</p> Signup and view all the answers

    What is a characteristic of IDH-wildtype glioblastomas?

    <p>Incomplete/partial deletions on either chromosome arm</p> Signup and view all the answers

    What is a common feature of IDH-mutant and 1p/19q-codeleted oligodendrogliomas?

    <p>Presence of TERT promoter hotspot mutations</p> Signup and view all the answers

    What is a characteristic of IDH-mutant and 1p/19q-codeleted oligodendrogliomas arising in teenagers?

    <p>Lack of TERT promoter mutation</p> Signup and view all the answers

    What is the assumed timing of TERT promoter mutation in IDH-mutant and 1p/19q-codeleted oligodendrogliomas?

    <p>Early event</p> Signup and view all the answers

    What is a common feature of both familial and sporadic gliomas?

    <p>Shared genomic landscapes</p> Signup and view all the answers

    Which gene is associated with increased risk of oligodendroglioma?

    <p>ERCC2</p> Signup and view all the answers

    What is a characteristic of familial oligodendroglioma?

    <p>Germline mutations of the POT1 gene</p> Signup and view all the answers

    What is a common feature of oligodendrogliomas and IDH-mutant astrocytomas?

    <p>Production of 2-hydroxyglutarate</p> Signup and view all the answers

    Which variant is associated with IDH-mutant gliomas?

    <p>rs5839764</p> Signup and view all the answers

    What is a characteristic of IDH-mutant, TERT promoter-mutant, and 1p/19q-codeleted oligodendrogliomas?

    <p>Variants in the FAM20C gene</p> Signup and view all the answers

    Which gene is associated with familial oligodendroglioma with 1p/19q codeletion?

    <p>POT1</p> Signup and view all the answers

    What is the function of the D2HGDH gene?

    <p>Codes for D-2-hydroxyglutarate dehydrogenase</p> Signup and view all the answers

    Study Notes

    Oligodendroglioma, IDH-mutant and 1p/19q-codeleted

    • Definition: A diffusely infiltrating glioma with IDH1 or IDH2 mutation and codeletion of chromosome arms 1p and 19q (CNS WHO grade 2 or 3)

    ICD-O Coding

    • 9450/3: Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, grade 2
    • 9451/3: Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, grade 3

    ICD-11 Coding

    • 2A00.0Y & XH7K31: Other specified gliomas of brain & Oligodendroglioma, IDH-mutant and 1p/19q-codeleted

    Localization

    • 59% of oligodendrogliomas are located in the frontal lobe, 14% in the temporal lobe, 10% in the parietal lobe, and 1% in the occipital lobe
    • 62% of CNS WHO grade 3 oligodendrogliomas are frontal tumors, 16% are temporal, 15% are parietal, and 6% are occipital

    Genetic Profile

    • Entity-defining alterations: missense mutations affecting IDH1 codon 132 or IDH2 codon 172 combined with whole-arm deletions of 1p and 19q
    • Over 90% of IDH mutations in oligodendrogliomas correspond to the canonical IDH1 p.R132H mutation
    • The 1p/19q codeletion is caused by an unbalanced translocation between chromosomes 1 and 19, resulting in loss of the der(1;19) (p10;q10) chromosome
    • TERT promoter hotspot mutations are present in the vast majority of IDH-mutant and 1p/19q-codeleted oligodendrogliomas

    Germline Mutations

    • Familial oligodendrogliomas have been associated with germline mutations of POT1, a shelterin complex gene
    • Cases of familial oligodendroglioma with 1p/19q codeletion have been reported
    • Variants in or near the D2HGDH gene, which codes for D-2-hydroxyglutarate dehydrogenase, have been associated with IDH-mutant gliomas
    • rs111976262, located near the FAM20C gene, has been associated with IDH-mutant, TERT promoter-mutant, and 1p/19q-codeleted oligodendrogliomas

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    Description

    A type of diffusely infiltrating glioma with IDH1 or IDH2 mutation and codeletion of chromosome arms 1p and 19q, classified as CNS WHO grade 2 or 3.

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