loading...| Preferred Name | Pleomorphic xanthoastrocytoma | |
| Synonyms |
PXA |
|
| Definitions |
A rare low-grade astrocytoma characterized by superficial location in the cerebral hemispheres with involvement of the meninges, composed of GFAP-expressing cells showing nuclear and cytoplasmic pleomorphism and xanthomatous change, surrounded by a reticulin network. The tumor corresponds to WHO grade II and typically affects children and young adults, who often present with a long history of seizures. Extent of resection and mitotic index are important prognostic factors. |
|
| ID |
http://www.orpha.net/ORDO/Orphanet_251607 |
|
| alternative_term |
PXA |
|
| definition |
A rare low-grade astrocytoma characterized by superficial location in the cerebral hemispheres with involvement of the meninges, composed of GFAP-expressing cells showing nuclear and cytoplasmic pleomorphism and xanthomatous change, surrounded by a reticulin network. The tumor corresponds to WHO grade II and typically affects children and young adults, who often present with a long history of seizures. Extent of resection and mitotic index are important prognostic factors. |
|
| definition_citation |
Orphanet |
|
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=251607 |
|
| has_age_of_onset |
All ages |
|
| has_inheritance |
Not applicable |
|
| hasDbXref |
ICD-11:2A00.0Y ICD-11:XH99U2 ICD-10:C71.9 UMLS:C0334586 |
|
| label |
Pleomorphic xanthoastrocytoma |
|
| notation |
ORPHA:251607 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_251607 |
|
| prefLabel |
Pleomorphic xanthoastrocytoma |
|
| 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 |