Abstract
Oligodendroglioma (WHO grade II) is a diffusely infiltrating, slow-growing glioma with IDH1 or IDH2 mutation and co-deletion of chromosomal arms 1p and 19q. It affects adults (mean age: 40–45 years) and involves the cortex and white matter of the cerebral hemispheres (frontal lobe in 50–65% of all cases).
Radiologically, it is a well-defined infiltrative mass; the involved cortex appears enlarged, and calcifications are common.
Histologically, the tumor is composed of monomorphic cells with round nuclei, perinuclear halo only seen on paraffin sections (honeycomb appearance), and moderate cellularity.
Treatment consists in surgical removal of the lesion. Mean survival is 11.6 years. Factors accounting for favorable outcome include young age, frontal localization, postoperative Karnofsky score, lack of contrast enhancement, and macroscopically complete surgical removal.
Anaplastic oligodendroglioma (WHO grade III) is an IDH-mutant and 1p/19q co-deleted oligodendroglioma with focal or diffuse histological features of anaplasia (in particular, pathological microvascular proliferation and/or brisk mitotic activity).
Histologically, it is a cellular, diffusely infiltrating tumor with marked cellular and nuclear pleomorphism, composed of cells reminiscent of oligodendrocytes with round hyperchromatic nuclei, perinuclear halo, and scant cellular processes.
Treatment consists of surgery, radiotherapy, and chemotherapy (PCV regimen: procarbazine, CCNU, vincristine). Clinical outcome shows a median survival time 4–5 years.
The most commonly detected genomic aberration in oligodendrogliomas is a heterozygous loss (LOH) of the short arm of chromosome 1 associated with LOH of the long arm of chromosome 19 (1p/19q co-deletion). Mutations in the IDH1- and IDH2 genes (encoding isocitrate dehydrogenase 1 and -2, respectively) are another characteristic feature in oligodendrogliomas. Other changes include point mutations in the CIC (capicua transcriptional repressor) gene and in the FUBP1 (far upstream element-binding protein 1) gene on chromosome 1p31.1. Several genes (MGMT, CDKN2A, DAPK1, RB1, ESR1) were shown to be affected by promoter hypermethylation, resulting in reduced expression levels. Elevated expression levels of platelet-derived growth factor and platelet-derived growth factor receptor were described.
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Weis, S. et al. (2019). Oligodendroglioma WHO Grade II-Anaplastic Oligodendroglioma WHO Grade III. In: Imaging Brain Diseases. Springer, Vienna. https://doi.org/10.1007/978-3-7091-1544-2_58
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