Oligodendroglioma grade 3

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Oligodendroglioma grade 3

ORPHA:251630Disease

Also called Anaplastic oligodendroglioma

What it is

A rare glial tumor characterized by a grade III oligodendroglial tumour with focal or diffuse anaplastic features. It typically occurs in the supratentorial white matter. Histologically, the cells are enlarged and epithelioid with pleomorphic and increased size nuclei, a vesicular chromatin pattern and prominent nucleoli. Most patients present with seizures.

Key facts

Prevalence
<1 / 1 000 000 (annual incidence, Europe)
Age of onset
Adult
Inheritance
Not applicable
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes

POT1Major susceptibility factor
IDH2Biomarker tested

ICD-10 codes

C71.9filed under a broader ICD-10 category — shared with 26 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 9472MEDDRA 10026659MONDO 0016696OMIM 137800UMLS C0334590

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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