Gliomas Diagnostic Criteria and Prognosis
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In IDH-mutant and 1p/19q-codeleted oligodendrogliomas, what is associated with shorter PFS and OS?

  • Low mitotic count (< 2.5 mitoses/mm^2^)
  • Presence of necrosis
  • High mitotic count (≥ 2.5 mitoses/mm^2^) (correct)
  • Absence of microvascular proliferation

    • What is a significant prognostic factor in cases lacking CDKN2A homozygous deletion?

  • High mitotic count
  • Microvascular proliferation and/or necrosis (correct)
  • 1p/19q codeletion
  • IDH mutation

    • What is an independent predictor of survival for patients with oligodendroglial tumours in older studies?

  • Mitotic count
  • CNS WHO grade (correct)
  • IDH mutation
  • 1p/19q codeletion

    • In patients with gliomas with concurrent IDH mutation and TERT promoter mutation, what is associated with longer survival times?

    <p>Grade 2 tumours</p> Signup and view all the answers

    What is associated with shorter OS in patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas?

    <p>CNS WHO grade 3</p> Signup and view all the answers

    What percentage of older histological series correspond to IDH-mutant and 1p/19q-codeleted oligodendrogliomas?

    <p>30-80%</p> Signup and view all the answers

    What is the median survival time of patients with CNS WHO grade 3 IDH-mutant and 1p/19q-codeleted oligodendroglioma treated with radiotherapy and PCV chemotherapy?

    <p>≥ 14 years</p> Signup and view all the answers

    What is the typical pattern of recurrence in oligodendrogliomas?

    <p>Local recurrence with occasional leptomeningeal spread</p> Signup and view all the answers

    What is a clinical factor associated with a more favourable outcome in oligodendroglioma?

    <p>Presentation with seizures</p> Signup and view all the answers

    What is the significance of IDH mutation and 1p/19q codeletion in oligodendrogliomas?

    <p>Associated with favourable response to therapy and longer survival</p> Signup and view all the answers

    What is the characteristic of malignant progression in oligodendroglioma compared to IDH-mutant astrocytoma?

    <p>Slower progression in oligodendroglioma</p> Signup and view all the answers

    What is the predictive value of higher tumour mutation burden in patients with IDH-mutant gliomas?

    <p>Shorter survival</p> Signup and view all the answers

    Which genetic mutation is associated with poor prognosis in oligodendrogliomas?

    <p>CIC mutation</p> Signup and view all the answers

    What is the impact of CDK4 amplification on outcome in oligodendrogliomas?

    <p>No impact on outcome</p> Signup and view all the answers

    What is the typical treatment approach for patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas of CNS WHO grade 2?

    <p>Deferred radiotherapy and chemotherapy until tumour progression</p> Signup and view all the answers

    What is the outcome of the EORTC 22845 trial in patients with progressive CNS WHO grade 2 gliomas?

    <p>Radiotherapy prolonged PFS but not OS</p> Signup and view all the answers

    What is the benefit of radiotherapy plus PCV chemotherapy in patients with CNS WHO grade 3 oligodendrogliomas?

    <p>Improved OS</p> Signup and view all the answers

    What is the genetic hallmark of oligodendrogliomas?

    <p>1p/19q codeletion</p> Signup and view all the answers

    What is the primary concern with therapy in patients with IDH-mutant oligodendrogliomas?

    <p>Therapy-associated neurotoxicity</p> Signup and view all the answers

    What is associated with shorter PFS and OS in IDH-mutant and 1p/19q-codeleted CNS WHO grade 3 oligodendrogliomas?

    <p>High mitotic count</p> Signup and view all the answers

    In cases lacking CDKN2A homozygous deletion, what is of prognostic significance?

    <p>Presence of microvascular proliferation and/or necrosis</p> Signup and view all the answers

    What is associated with longer survival times in patients with gliomas with concurrent IDH mutation and TERT promoter mutation?

    <p>Grade 2 tumours</p> Signup and view all the answers

    What is an independent predictor of survival for patients with oligodendroglial tumors in older studies?

    <p>CNS WHO grade</p> Signup and view all the answers

    In patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas, what is associated with shorter OS?

    <p>CNS WHO grade 3</p> Signup and view all the answers

    What is the significance of CNS WHO grade in patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas?

    <p>It is an independent predictor of survival</p> Signup and view all the answers

    What is associated with favourable response to therapy and median survival times of > 10 years in oligodendrogliomas?

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

    What is the characteristic pattern of recurrence in oligodendrogliomas?

    <p>Local recurrence with rare malignant progression</p> Signup and view all the answers

    What is a clinical factor associated with a more favourable outcome in oligodendrogliomas?

    <p>Presentation with seizures</p> Signup and view all the answers

    What is the significance of IDH mutation and 1p/19q codeletion in oligodendrogliomas?

    <p>They are characteristic genetic hallmarks of oligodendrogliomas</p> Signup and view all the answers

    What percentage of older histological series correspond to IDH-mutant and 1p/19q-codeleted oligodendrogliomas?

    <p>30-80%</p> Signup and view all the answers

    How does the malignant progression in oligodendrogliomas compare to IDH-mutant astrocytoma?

    <p>It takes longer in oligodendrogliomas</p> Signup and view all the answers

    What is associated with shorter survival in patients with IDH-mutant gliomas, including oligodendrogliomas?

    <p>Higher tumour mutation burden</p> Signup and view all the answers

    What is the significance of CIC mutation in oligodendrogliomas?

    <p>It is a marker of poor prognosis</p> Signup and view all the answers

    What is the typical treatment approach for patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas of CNS WHO grade 2?

    <p>Deferred radiotherapy and chemotherapy until tumour progression</p> Signup and view all the answers

    What is the outcome of the EORTC 22845 trial in patients with progressive CNS WHO grade 2 gliomas?

    <p>Adjuvant radiotherapy prolonged PFS but not OS</p> Signup and view all the answers

    What is the benefit of radiotherapy plus PCV chemotherapy in patients with CNS WHO grade 3 oligodendrogliomas?

    <p>It improves OS but not PFS</p> Signup and view all the answers

    What is the primary concern with therapy in patients with IDH-mutant oligodendrogliomas?

    <p>Therapy-associated neurotoxicity</p> Signup and view all the answers

    What is the impact of RB1 homozygous deletion on outcome in oligodendrogliomas?

    <p>It has no impact on outcome</p> Signup and view all the answers

    Study Notes

    Staging and Prognosis

    • IDH-mutant and 1p/19q-codeleted oligodendrogliomas are associated with favourable response to therapy and median survival times of > 10 years.
    • Patients with CNS WHO grade 3 IDH-mutant and 1p/19q-codeleted oligodendroglioma who participated in prospective clinical trials and were treated with a combination of radiotherapy and procarbazine, lomustine, and vincristine (PCV) chemotherapy showed a median survival of ≥ 14 years.

    Prognostic Factors

    • Younger patient age at diagnosis, frontal lobe location, presentation with seizures, high postoperative Karnofsky score, and macroscopically complete surgical removal are associated with a more favourable outcome.
    • High mitotic count (≥ 2.5 mitoses/mm^2) is linked to shorter PFS and OS in IDH-mutant and 1p/19q-codeleted CNS WHO grade 3 oligodendrogliomas.
    • The presence of microvascular proliferation and/or necrosis is of prognostic significance in cases lacking CDKN2A homozygous deletion.

    CNS WHO Grading

    • Older studies reported CNS WHO grade as an independent predictor of survival for patients with oligodendroglial tumours.
    • Recent studies have shown that patients with grade 2 tumours had longer survival times than those with grade 3 tumours.

    Treatment

    • The optimal postoperative treatment of patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas of CNS WHO grade 2 is a matter of ongoing discussion.
    • Radiotherapy and chemotherapy are often deferred until tumour progression in patients with CNS WHO grade 2 tumours.
    • Patients with symptomatic and progressive tumours, with CNS WHO grade 3 tumours, or with large residual tumours after surgery usually receive immediate further treatment with radiotherapy and/or chemotherapy.
    • Adjuvant radiotherapy prolonged PFS but not OS in patients with progressive CNS WHO grade 2 gliomas.
    • Long-term follow-up data from randomized trials showed a major increase in OS after radiotherapy plus PCV chemotherapy in patients with CNS WHO grade 3 oligodendrogliomas.

    Staging and Prognosis

    • IDH-mutant and 1p/19q-codeleted oligodendrogliomas are associated with favourable response to therapy and median survival times of > 10 years.
    • Patients with CNS WHO grade 3 IDH-mutant and 1p/19q-codeleted oligodendroglioma who participated in prospective clinical trials and were treated with a combination of radiotherapy and procarbazine, lomustine, and vincristine (PCV) chemotherapy showed a median survival of ≥ 14 years.

    Prognostic Factors

    • Younger patient age at diagnosis, frontal lobe location, presentation with seizures, high postoperative Karnofsky score, and macroscopically complete surgical removal are associated with a more favourable outcome.
    • High mitotic count (≥ 2.5 mitoses/mm^2) is linked to shorter PFS and OS in IDH-mutant and 1p/19q-codeleted CNS WHO grade 3 oligodendrogliomas.
    • The presence of microvascular proliferation and/or necrosis is of prognostic significance in cases lacking CDKN2A homozygous deletion.

    CNS WHO Grading

    • Older studies reported CNS WHO grade as an independent predictor of survival for patients with oligodendroglial tumours.
    • Recent studies have shown that patients with grade 2 tumours had longer survival times than those with grade 3 tumours.

    Treatment

    • The optimal postoperative treatment of patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas of CNS WHO grade 2 is a matter of ongoing discussion.
    • Radiotherapy and chemotherapy are often deferred until tumour progression in patients with CNS WHO grade 2 tumours.
    • Patients with symptomatic and progressive tumours, with CNS WHO grade 3 tumours, or with large residual tumours after surgery usually receive immediate further treatment with radiotherapy and/or chemotherapy.
    • Adjuvant radiotherapy prolonged PFS but not OS in patients with progressive CNS WHO grade 2 gliomas.
    • Long-term follow-up data from randomized trials showed a major increase in OS after radiotherapy plus PCV chemotherapy in patients with CNS WHO grade 3 oligodendrogliomas.

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    Description

    This quiz covers the essential and desirable diagnostic criteria for gliomas, as well as prognosis and prediction based on survival data and molecular stratification.

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