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Orphanet Rare Disease Ontology
Last uploaded:
December 4, 2024
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| Id | http://www.orpha.net/ORDO/Orphanet_251618
http://www.orpha.net/ORDO/Orphanet_251618
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|---|---|
| Preferred Name | Subependymal giant cell astrocytoma |
| Definitions |
A rare low-grade astrocytoma characterized by a benign, slowly growing lesion typically arising in the wall of the lateral ventricles, composed of large ganglioid astrocytes. The tumor corresponds to WHO grade I and typically occurs during the first two decades of life in patients with tuberous sclerosis complex. Most patients present with worsening of epilepsy or symptoms of increased intracranial pressure.
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| Synonyms |
SEGA
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| Type | http://www.w3.org/2002/07/owl#Class |
All Properties
| definition_citation | Orphanet |
|---|---|
| definition | A rare low-grade astrocytoma characterized by a benign, slowly growing lesion typically arising in the wall of the lateral ventricles, composed of large ganglioid astrocytes. The tumor corresponds to WHO grade I and typically occurs during the first two decades of life in patients with tuberous sclerosis complex. Most patients present with worsening of epilepsy or symptoms of increased intracranial pressure. |
| label |
Subependymal giant cell astrocytoma
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| prefLabel |
Subependymal giant cell astrocytoma
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| hasDbXref |
ICD-11:2A00.0Y
UMLS:C0205768
ICD-10:D43.2
ICD-11:XH1L48
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| notation |
ORPHA:251618
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| alternative_term |
SEGA
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| part_of | |
| prefixIRI |
ORDO:Orphanet_251618
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| expertlink | |
| subClassOf | |
| type | |
| treeView |
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