Oligodendroglioma
Oligodendroglioma is a slow-growing diffuse glioma arising from the brain's myelin-producing oligodendrocytes. According to the 2021 World Health Organization (WHO) classification, the diagnosis requires the combined presence of an IDH mutation and 1p/19q co-deletion. It has the most favorable prognosis among diffuse gliomas.
Ostatnia aktualizacja: 2026-06-06
Definition
Oligodendroglioma is a diffuse infiltrative brain tumor that develops from oligodendrocytes, the cells that produce the myelin sheathing nerve fibers. It accounts for roughly 5% of all primary intracranial tumors and is usually located in the white matter of the cerebral hemispheres, most often the frontal lobe.
Epidemiology
Oligodendroglioma typically occurs in young-to-middle-aged adults aged 35-50 years and is slightly more common in men. The large majority are supratentorial. Most are sporadic with no identifiable risk factor. Because of its cortical location, many patients present with epilepsy as the sole symptom for a long period.
Symptoms
The most common presenting symptom is epileptic seizures, which may be the only finding for years. As the tumor grows, slowly progressive headache, personality and behavioral changes with frontal lobe involvement, memory and executive dysfunction, and location-dependent limb weakness, incoordination or speech difficulty (aphasia) may appear. Because of calcification and increased vascularity, the bleeding risk is somewhat higher than in other gliomas.
Diagnosis
MRI is the principal imaging method, typically showing a cortical-subcortical lesion that is hyperintense on T2/FLAIR. Calcification, which is common on CT, aids the diagnosis. Definitive diagnosis is made by pathological and molecular examination of surgically obtained tissue. Under the 2021 WHO classification, demonstration of both an IDH1/IDH2 mutation and 1p/19q co-deletion is mandatory for the diagnosis; without these tests, oligodendroglioma cannot be diagnosed.
Classification and Grading (2021 WHO CNS5)
The 2021 WHO classification based the diagnosis of oligodendroglioma on molecular criteria. IDH-mutant tumors without 1p/19q co-deletion now fall into the astrocytoma category. The term 'anaplastic oligodendroglioma' has been removed; the tumor is classified only as grade 2 or grade 3, and there is no grade 4 oligodendroglioma. 1p/19q co-deletion is both a diagnostic criterion and a strong indicator of chemosensitivity and favorable prognosis.
Treatment Options
Treatment is planned according to grade, extent of resection, molecular profile, age and the patient's condition. The first step is the widest safe surgical resection; awake craniotomy and cortical mapping are used for tumors near eloquent (functionally critical) areas. For low-grade cases, options include close surveillance, the brain-penetrant IDH inhibitor vorasidenib (approved for certain grade 2 cases after surgery), and radiotherapy with chemotherapy when needed. For grade 3 cases, the standard approach is radiotherapy combined with chemotherapy (PCV or temozolomide); the PCV regimen is supported by long-term follow-up data.
Prognosis
Thanks to 1p/19q co-deletion and IDH mutation, oligodendroglioma has the most favorable prognosis among diffuse gliomas and responds to radiotherapy and chemotherapy. Prognosis varies with age, extent of resection, tumor grade and molecular features. Because low-grade tumors may transform to higher grade over time, close follow-up is recommended. Outcomes vary individually and no outcome can be guaranteed.
Źródła
- Greenberg MS. Greenberg's Handbook of Neurosurgery. 10th ed. Thieme; 2023:662-666.
- Osborn AG, Hedlund GL, Salzman KL. Osborn's Brain: Imaging, Pathology, and Anatomy. 2nd ed. Elsevier; 2018:557-558.
- Louis DN, et al. The 2021 WHO Classification of Tumors of the Central Nervous System. Neuro Oncol. 2021.
- Weller M, et al. EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood. Nat Rev Clin Oncol. 2021.
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