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Astrocytoma and oligodendroglioma

What's new

The American Society of Clinical Oncology (ASCO) and the Society for Neuro-Oncology (SNO) have released a rapid recommendation update for the management of astrocytoma and oligodendroglioma. Based on the INDIGO trial results and subsequent FDA approval, vorasidenib is now recommended for patients with IDH-mutant, WHO grade 2 oligodendroglioma (1p/19q co-deleted) or astrocytoma (1p/19q non-co-deleted), in whom further treatment with radiotherapy and chemotherapy has been or can be deferred following one or more surgeries. .

Background

Overview

Definition
Astrocytomas and oligodendrogliomas are malignant primary brain tumors originating from astrocytes and oligodendrocytes, respectively. Most cases are classified as WHO grade 2 or 3 gliomas and have an IDH mutation.
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Pathophysiology
Mutations in the IDH1 and IDH2 genes lead to reduced α-KG levels in glial cells and increase DNA methylation, resulting in pro-tumor consequences. Other comon genetic alterations include CDKN2A/CDKN2B deletion in astrocytomas and 1p19q co-deletion in oligodendrogliomas.
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Epidemiology
The incidence of diffuse astrocytomas and oligodendrogliomas in the US is estimated at 0.44 and 0.23 per 100,000 person-year, respectively.
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Risk factors
Risk factors for primary brain tumors include Ionizing radiation, such as head and neck radiotherapy and CT in childhood, cancer-predisposing syndromes, and a family history of brain tumors.
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Disease course
The clinical course of astrocytomas is generally more progressive, whereas oligodendrogliomas have a relatively favorable prognosis compared to other malignant gliomas. Primary brain tumors typically present with various focal neurologic deficits, depending on tumor location, along with general neurological symptoms, such as headaches, seizures, fatigue, and increased ICP.
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Prognosis and risk of recurrence
Astrocytomas and oligodendrogliomas have the potential to transform into higher-grade tumors, including glioblastoma. The 5-year survival rates for diffuse astrocytomas and diffuse oligodendrogliomas are estimated at 53.5% and 84.6%, respectively.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of astrocytoma and oligodendroglioma are prepared by our editorial team based on guidelines from the American Association of Neurological Surgeons (AANS/CNS 2025,2015), the Congress of Neurological Surgeons (CNS 2025), the Society for Neuro-Oncology (SNO/ASCO 2025,2022), the American Society for Radiation Oncology (ASTRO 2022), the European Association of Neuro-Oncology (EANO 2021,2017), the ...
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Screening and diagnosis

Indications for screening: as per EANO 2021 guidelines, recognize that screening and prevention have no major role for patients with gliomas.
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Classification and risk stratification

Classification: as per EANO 2021 guidelines, use the most recent WHO Classification of Tumors of the CNS for glioma classification, complemented by cIMPACT-NOW.
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Diagnostic investigations

Diagnostic imaging
As per EANO 2021 guidelines:
Obtain MRI without and with gadolinium contrast as the first choice diagnostic imaging modality.
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Suspect pseudoprogression in patients with an increase of abnormalities on neuroimaging in the first months after local therapeutic interventions, including radiotherapy, and after experimental local treatments.
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  • Evaluation of complications

Diagnostic procedures

Biopsy: as per EANO 2021 guidelines, perform resection as feasible or biopsy followed by involved-field radiotherapy and maintenance procarbazine, lomustine, and vincristine polychemotherapy in patients with IDH-mutant astrocytomas, WHO grade 2 requiring further treatment.
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  • Histopathology

Medical management

General principles: as per EANO 2021 guidelines, take into account the Karnofsky performance score, neurological function, age, and individual risks and benefits for clinical decision-making.
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Karnofsky Performance Scale
Patient description
Normal, no complaints, no evidence of disease
Able to carry on normal activity, minor signs or symptoms of disease
Normal activity with effort, some signs or symptoms of disease
Cares for self, unable to carry on normal activity or to do active work
Requires occasional assistance but is able to care for most personal needs
Requires considerable assistance and frequent medical care
Disabled, requires special care and assistance
Severely disabled, hospital admission is indicated, death not imminent
Very sick, hospital admission necessary, active supportive treatment necessary
Moribund
Dead
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  • Watchful waiting

  • Chemoradiotherapy (general principles)

  • Chemoradiotherapy (astrocytoma, grade 2)

  • Chemoradiotherapy (astrocytoma, grade 3)

  • Chemoradiotherapy (oligodendroglioma, grade 2)

  • Chemoradiotherapy (oligodendroglioma, grade 3)

  • Immunotherapy and targeted therapy

  • Seizure prophylaxis

  • Thromboprophylaxis

  • P. jirovecii prophylaxis

  • Management of complications (headache)

  • Management of complications (seizure)

  • Management of complications (cerebral edema)

  • Management of complications (psychological and cognitive disorders)

  • Management of complications (delirium)

  • Management of complications (fatigue)

  • Management of complications (VTE)

Therapeutic procedures

General principles of radiotherapy, therapeutic aims: as per AANS/CNS 2015 guidelines, offer radiotherapy to prolong progression-free survival, irrespective of the extent of resection, in adult patients with newly diagnosed low-grade glioma.
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  • General principles of radiotherapy (prognostic factors)

  • Technical considerations for radiotherapy (techniques and field design)

  • Technical considerations for radiotherapy (dosing)

Perioperative care

Preoperative imaging: as per AANS/CNS 2015 guidelines, obtain preoperative functional MRI and diffusion tensor imaging in the appropriate clinical setting to improve functional outcomes after surgery for low-grade gliomas.
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Surgical interventions

Surgical resection: as per CNS 2025 guidelines, consider performing surgical resection over observation or biopsy in adult patients with imaging suggestive of WHO grade II diffuse glioma (oligodendroglioma or astrocytoma) to improve overall survival.
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  • Intraoperative imaging

Specific circumstances

Elderly patients: as per EANO 2021 guidelines, offer radiotherapy (such as 2.66 Gy for 15 sessions) or chemotherapy with temozolomide (5 out of 28 days) based on MGMT promoter methylation status in elderly patients with IDH-wild-type glioblastoma ineligible for combined chemoradiotherapy.
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Patient education

Genetic counseling: as per EANO 2021 guidelines, offer genetic counseling to patients with relevant germline variants or suspected hereditary cancer syndromes and subsequently refer for molecular genetic testing.
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Follow-up and surveillance

Evaluation for recurrence: as per AANS/CNS 2025 guidelines, consider obtaining advanced imaging, such as MRS, perfusion-weighted imaging, DWI, or PET, to identify tumor recurrence or histologic progression in adult patients with suspected recurrence of histologically proven WHO grade 2 diffuse glioma.
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  • Management of recurrence