Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Gliomatosis cerebri
Synonyms
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.

ID

http://www.orpha.net/ORDO/Orphanet_251582

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.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=251582

has_age_of_onset

Adult

has_inheritance

Not applicable

hasDbXref

UMLS:C0334576

ICD-11:2A00.0Y

ICD-10:C71.0

MedDRA:10066254

ICD-11:XH6ZH4

label

Gliomatosis cerebri

notation

ORPHA:251582

part_of

http://www.orpha.net/ORDO/Orphanet_251561

prefixIRI

ORDO:Orphanet_251582

prefLabel

Gliomatosis cerebri

present_in

Europe AND has_annual_incidence_average_value : 0.01 AND has_annual_incidence_range : <1 / 1 000 000

treeView

http://www.orpha.net/ORDO/Orphanet_251561

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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