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CNS Oncol. 2015;4(5):287-94. doi: 10.2217/cns.15.32. Epub 2015 Nov 6.

Molecular background of oligodendroglioma: 1p/19q, IDH, TERT, CIC and FUBP1.

Author information

1
Department of Neurosurgery & Translational Neuro-Oncology Laboratory, Harvard Medical School, Boston, MA 02115, USA.
2
Massachusetts General Hospital Cancer Center, Harvard Medical School, 32 Fruit Street - Yawkey 9E, Boston, MA 02114, USA.
3
Department of Pathology, Harvard Medical School, Boston, MA 02115, USA.
4
Southern Alberta Cancer Research Institute, University of Calgary, Calgary, AB T2N 1N4, Canada.
5
Hotchkiss Brain Institute, University of Calgary, Calgary, AB T2N 1N4, Canada.

Abstract

Oligodendroglioma is the quintessential molecularly-defined brain tumor. The characteristic whole-arm loss of the long arm of chromosome 1 and the short arm of chromosome 19 (1p/19q-codeletion) within the genome of these tumors facilitated the reproducible molecular identification of this subcategory of gliomas. More recently, recurrent molecular genetic alterations have been identified to occur concurrently with 1p/19q-codeletion, and definitively identify these tumors, including mutations in IDH1/2, CIC, FUBP1, and the TERT promoter, as well as the absence of ATRX and TP53 alterations. These findings provide a foundation for the consistent diagnosis of this tumor type, upon which a generation of clinical investigators have assembled a strong evidence base for the effective treatment of this disease with radiation and chemotherapy.

KEYWORDS:

1p/19q loss; CIC mutation; FUBP1 mutation; IDH mutation; TERT mutation; oligodendroglioma

PMID:
26545048
PMCID:
PMC6082339
DOI:
10.2217/cns.15.32
[Indexed for MEDLINE]
Free PMC Article

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