38 Questions
In IDH-mutant and 1p/19q-codeleted oligodendrogliomas, what is associated with shorter PFS and OS?
High mitotic count (≥ 2.5 mitoses/mm^2^)
What is a significant prognostic factor in cases lacking CDKN2A homozygous deletion?
Microvascular proliferation and/or necrosis
What is an independent predictor of survival for patients with oligodendroglial tumours in older studies?
CNS WHO grade
In patients with gliomas with concurrent IDH mutation and TERT promoter mutation, what is associated with longer survival times?
Grade 2 tumours
What is associated with shorter OS in patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas?
CNS WHO grade 3
What percentage of older histological series correspond to IDH-mutant and 1p/19q-codeleted oligodendrogliomas?
30-80%
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?
≥ 14 years
What is the typical pattern of recurrence in oligodendrogliomas?
Local recurrence with occasional leptomeningeal spread
What is a clinical factor associated with a more favourable outcome in oligodendroglioma?
Presentation with seizures
What is the significance of IDH mutation and 1p/19q codeletion in oligodendrogliomas?
Associated with favourable response to therapy and longer survival
What is the characteristic of malignant progression in oligodendroglioma compared to IDH-mutant astrocytoma?
Slower progression in oligodendroglioma
What is the predictive value of higher tumour mutation burden in patients with IDH-mutant gliomas?
Shorter survival
Which genetic mutation is associated with poor prognosis in oligodendrogliomas?
CIC mutation
What is the impact of CDK4 amplification on outcome in oligodendrogliomas?
No impact on outcome
What is the typical treatment approach for patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas of CNS WHO grade 2?
Deferred radiotherapy and chemotherapy until tumour progression
What is the outcome of the EORTC 22845 trial in patients with progressive CNS WHO grade 2 gliomas?
Radiotherapy prolonged PFS but not OS
What is the benefit of radiotherapy plus PCV chemotherapy in patients with CNS WHO grade 3 oligodendrogliomas?
Improved OS
What is the genetic hallmark of oligodendrogliomas?
1p/19q codeletion
What is the primary concern with therapy in patients with IDH-mutant oligodendrogliomas?
Therapy-associated neurotoxicity
What is associated with shorter PFS and OS in IDH-mutant and 1p/19q-codeleted CNS WHO grade 3 oligodendrogliomas?
High mitotic count
In cases lacking CDKN2A homozygous deletion, what is of prognostic significance?
Presence of microvascular proliferation and/or necrosis
What is associated with longer survival times in patients with gliomas with concurrent IDH mutation and TERT promoter mutation?
Grade 2 tumours
What is an independent predictor of survival for patients with oligodendroglial tumors in older studies?
CNS WHO grade
In patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas, what is associated with shorter OS?
CNS WHO grade 3
What is the significance of CNS WHO grade in patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas?
It is an independent predictor of survival
What is associated with favourable response to therapy and median survival times of > 10 years in oligodendrogliomas?
IDH-mutant and 1p/19q-codeleted oligodendrogliomas
What is the characteristic pattern of recurrence in oligodendrogliomas?
Local recurrence with rare malignant progression
What is a clinical factor associated with a more favourable outcome in oligodendrogliomas?
Presentation with seizures
What is the significance of IDH mutation and 1p/19q codeletion in oligodendrogliomas?
They are characteristic genetic hallmarks of oligodendrogliomas
What percentage of older histological series correspond to IDH-mutant and 1p/19q-codeleted oligodendrogliomas?
30-80%
How does the malignant progression in oligodendrogliomas compare to IDH-mutant astrocytoma?
It takes longer in oligodendrogliomas
What is associated with shorter survival in patients with IDH-mutant gliomas, including oligodendrogliomas?
Higher tumour mutation burden
What is the significance of CIC mutation in oligodendrogliomas?
It is a marker of poor prognosis
What is the typical treatment approach for patients with IDH-mutant and 1p/19q-codeleted oligodendrogliomas of CNS WHO grade 2?
Deferred radiotherapy and chemotherapy until tumour progression
What is the outcome of the EORTC 22845 trial in patients with progressive CNS WHO grade 2 gliomas?
Adjuvant radiotherapy prolonged PFS but not OS
What is the benefit of radiotherapy plus PCV chemotherapy in patients with CNS WHO grade 3 oligodendrogliomas?
It improves OS but not PFS
What is the primary concern with therapy in patients with IDH-mutant oligodendrogliomas?
Therapy-associated neurotoxicity
What is the impact of RB1 homozygous deletion on outcome in oligodendrogliomas?
It has no impact on outcome
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.
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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