IDH-mutant, 1p/19q-codeleted oligodendrogliomas: Clinical features, pathology, and prognostic factors
- Martin van den Bent, MD, PhD
Martin van den Bent, MD, PhD
- Daniel den Hoed Oncology Center
- Neuro-Oncology Unit
- Section Editors
- Patrick Y Wen, MD
Patrick Y Wen, MD
- Section Editor — Neurooncology
- Professor of Neurology
- Harvard Medical School
- Jay S Loeffler, MD
Jay S Loeffler, MD
- Section Editor — Neurooncology
- Professor of Radiation Oncology
- Harvard Medical School
There are significant differences between oligodendrogliomas and other glial tumors in pathology, molecular pathogenesis, and natural history. These differences have important prognostic implications, which can affect treatment.
As of the 2016 update of the World Health Organization (WHO) classification of central nervous system tumors, oligodendroglial tumors are now more narrowly defined by molecular diagnostics to include only those diffuse gliomas harboring both a mutation in isocitrate dehydrogenase type 1 (IDH1) or type 2 (IDH2) and codeletion of chromosomes 1p and 19q . Mixed oligoastrocytoma no longer exists for fully characterized tumors, but the terminology is retained when referring to historically diagnosed tumors and for tumors with mixed oligoastrocytoma histology for which molecular testing is not available (ie, oligoastrocytoma not otherwise specified [NOS]). (See "Classification and pathologic diagnosis of gliomas".)
The clinical manifestations, classification, pathologic classification, and molecular prognostic factors associated with IDH-mutant, 1p/19q-codeleted oligodendroglioma and anaplastic oligodendroglioma will be reviewed here. The treatment of these tumors is discussed separately. (See "Management of low-grade glioma" and "Management of anaplastic oligodendroglial tumors".)
Oligodendroglial tumors are rare tumors, constituting approximately 5 percent of all neuroepithelial tumors of the central nervous system . Together, oligodendroglioma and anaplastic oligodendroglioma are one-tenth as common as glioblastoma, the most commonly occurring malignant primary brain tumor in adults. Approximately 1000 oligodendroglial tumors are diagnosed in the United States each year. (See "Incidence of primary brain tumors".)
Age of onset — Most oligodendrogliomas present in adults between 25 and 45 years of age. The median age at diagnosis is approximately 5 to 10 years older for World Health Organization (WHO) grade III (anaplastic) tumors compared with WHO grade II (low-grade) tumors. Oligodendrogliomas are occasionally diagnosed in teenagers and in adults over the age of 65 years.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- CLINICAL FEATURES
- Age of onset
- Signs and symptoms
- EVALUATION AND DIAGNOSIS
- Key molecular tests
- - IDH1/2 mutational testing
- - Loss of 1p and 19q
- - Additional molecular alterations
- Tumors previously called oligoastrocytoma
- Oligodendroglioma NOS
- PROGNOSIS AND PROGNOSTIC FACTORS
- Population-based estimates
- Clinical factors
- Tumor grade (II versus III)
- Molecular markers
- - 1p/19q-codeletion
- - IDH1/2 mutation
- - TERT promoter mutations
- Mechanism of chemosensitivity