Oligodendroglioma is a rare type of glioma, a tumor that develops from oligodendrocytes, the cells that produce the myelin sheath covering nerve fibers. These tumors are most often found in the frontal or temporal lobes of the brain. Oligodendrogliomas are characterized by specific genetic markers, most notably 1p/19q codeletion and IDH1/IDH2 mutations, which are now essential in confirming diagnosis and guiding treatment.
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What Is Oligodendroglioma?
Oligodendroglioma is a type of glioma, which is a brain tumor that grows in the brain tissue.
What are the grades of oligodendroglioma?
Oligodendrogliomas are typically grouped into two grades by the World Health Organization (WHO) 2021 classification:
- Grade II, low-grade oligodendroglioma — Slow-growing, often indolent tumors that may remain asymptomatic for years. Despite being low-grade, they can recur or progress over time.
- Grade III, also called anaplastic oligodendroglioma — Faster-growing, more aggressive tumors with a higher likelihood of recurrence and spread within the brain. These tumors require multimodal treatment with surgery, radiation, and chemotherapy.
How common is oligodendroglioma?
Oligodendrogliomas account for about 1% to 2% of all primary brain tumors and approximately 5% to 6% of gliomas. They are most common in young to middle-aged adults (ages 20 to 50), although they can occur at any age.
What causes oligodendroglioma?
Researchers aren’t sure what causes oligodendroglioma.
Oligodendroglioma risk factors
Although the exact cause is not fully understood, known risk factors for oligodendroglioma include:
- Family history of brain tumors — Rare but possible association.
- Genetic mutations — IDH1/IDH2 mutations and 1p/19q codeletion are defining features.
- Radiation exposure — Prior therapeutic head radiation may increase risk.
There is no evidence that lifestyle factors significantly contribute to risk.
Complications of oligodendroglioma
Complications may result from both the tumor and its treatment:
- Fatigue and cognitive decline.
- Neurological deficits — Speech, motor, or sensory impairment depending on tumor location.
- Seizures — Common presenting symptom.
- Side effects of treatment (radiation necrosis, chemotherapy-induced anemia, electrolyte imbalance, or infection).
- Tumor recurrence or progression.
How can I prevent oligodendroglioma?
There is nothing you can do to prevent oligodendroglioma.
What Are the Signs and Symptoms of Oligodendroglioma?
Oligodendroglioma symptoms may vary depending on the location and size of the tumor. The most common first symptom is a seizure.
Other symptoms may include:
- Balance or movement problems.
- Headaches.
- Nausea or vomiting.
- Personality changes.
- Speech, vision, or memory problems.
- Weakness or loss of sensation in your arms and/or legs.
When should I see a doctor about my oligodendroglioma symptoms?
If you have symptoms of oligodendroglioma, you should schedule an appointment with your doctor right away. If you have a seizure or experience other severe symptoms, you should dial 911 or go to the nearest hospital Emergency Department.
How Do You Diagnose Oligodendroglioma?
To diagnose oligodendroglioma, your doctor will ask about your symptoms. They'll do a physical exam and order imaging tests.
What to expect during your visit
If your doctor suspects an oligodendroglioma, they will:
- Ask about your symptoms.
- Perform a physical exam.
- Order imaging tests.
- Review your health history and medications.
Tests to diagnose oligodendroglioma
Diagnosis requires a combination of imaging, tissue analysis, and molecular testing:
- Biopsy or surgical resection — Confirms tumor type.
- CT scan — Useful in emergencies or for detecting calcifications, which are common in oligodendrogliomas.
- MRI with contrast — Preferred imaging to evaluate size, location, and tumor characteristics.
- Molecular testing — Essential to detect IDH mutations and 1p/19q codeletion, which guide prognosis and treatment choices.
Additional testing may include neurocognitive evaluations and seizure monitoring.
Oligodendroglioma prognosis
Prognosis varies depending on tumor grade, location, patient age, and genetic profile. Tumors with IDH mutations and 1p-19q codeletion have the best outcomes, often responding well to treatment.
- Five-year survival rate — About 79.5% overall.
- Median survival for anaplastic oligodendrogliomas — Three to eight years, depending on molecular profile and treatment.
- Median survival for low-grade oligodendrogliomas — Usually 10 to 15 years with appropriate therapy.
How Do You Treat Oligodendroglioma?
UPMC offers personalized treatment plans using a combination of therapies.
Treatment options for oligodendroglioma include:
Surgery
Surgery is the first-line treatment for oligodendroglioma whenever possible.
Minimally invasive surgery
Neuroendoport® surgery — A minimally invasive approach that uses a small port to access tumors through a dime-sized incision, reducing trauma, scarring, and recovery time.
Radiation therapy
- Used after surgery for the treatment of residual disease or recurrence.
- Can be delivered via external beam, stereotactic radiosurgery, or proton beam therapy.
Medical oncology
Medical oncology involves the use of various types of anticancer medications to target and destroy cancer cells.
Medical oncology treatments may include:
Chemotherapy
Chemotherapy uses medications that travel through your bloodstream and kill cancer cells throughout your body. Some types of chemotherapy medications can be placed directly at the site of the brain tumor.
The standard chemotherapy regimen is:
- PCV (procarbazine, lomustine, vincristine) or temozolomide.
- Choice depends on tumor genetics and patient tolerance.
Targeted therapy and immunotherapy
- IDH inhibitors are being studied in clinical trials.
- Immunotherapies such as checkpoint inhibitors are under investigation but not yet standard.
Clinical trials
Ongoing studies focus on improving survival and reducing side effects using novel agents and personalized therapy.
Treatment goals are to maximize tumor removal, control recurrence, and preserve neurological function. Medical oncology treatment may be given before or after surgery, or as a long-term treatment to reduce the risk of cancer recurrence.
Why Choose UPMC for Oligodendroglioma Care?
When you choose UPMC for oligodendroglioma care, you will receive:
- Access to world-class neurosurgery expertise — Our world-renowned experts treat the full spectrum of skull base and neurosurgical conditions using the latest diagnostic and treatment techniques.
- A full range of treatment options — We offer nonsurgical care and noninvasive, minimally invasive, and traditional open surgical procedures. This allows us to effectively treat all types of disorders while reducing your risk of complications.
- Multidisciplinary care — Our skull base surgeons collaborate with neurosurgeons, neurologists, radiologists, medical oncologists, rehabilitation specialists, and other medical experts to deliver comprehensive care that optimizes your recovery and quality of life.