loading...| Preferred Name |
Gliomatosis cerebri |
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| Synonyms |
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| Definitions |
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. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_251582 |
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| 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. |
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| definition_citation |
Orphanet |
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=251582 |
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| has_age_of_onset |
Adult |
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| has_inheritance |
Not applicable |
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| hasDbXref |
UMLS:C0334576 ICD-11:2A00.0Y ICD-10:C71.0 MedDRA:10066254 ICD-11:XH6ZH4 |
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| label |
Gliomatosis cerebri |
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| notation |
ORPHA:251582 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_251582 |
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| prefLabel |
Gliomatosis cerebri |
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| present_in |
Europe AND has_annual_incidence_average_value : 0.01 AND has_annual_incidence_range : <1 / 1 000 000 |
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| treeView | ||
| subClassOf |