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Gliomatosis cerebri
Disease definition
A rare glial tumor characterized by extensive infiltration of the brain, often extending to infratentorial structures and even the spinal cord. The tumor corresponds to WHO grade III and is composed of elongated glial cells typically resembling astrocytes. Cases in which the predominant cell type is oligodendroglial have also been described. Some tumors develop a circumscribed neoplastic mass in addition to the diffuse lesion, usually showing features of high-grade glioma. Clinical symptoms include dementia, headache, seizures, signs of increased intracranial pressure, and a variety of neurological deficits. Prognosis is generally poor.
ORPHA:251582
Classification level: Disorder- Synonym(s): -
- Prevalence: -
- Inheritance: Not applicable
- Age of onset: Adult
- ICD-10: C71.0
- OMIM: -
- UMLS: C0334576
- MeSH: -
- GARD: 6514
- MedDRA: 10066254
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