CNS5:Pleomorphic xanthoastrocytoma: Difference between revisions
No edit summary |
|||
| Line 245: | Line 245: | ||
For most PXAs, no inherited/familial cause is identified, and they are considered sporadic tumors. | For most PXAs, no inherited/familial cause is identified, and they are considered sporadic tumors. | ||
==Additional Information== | ==Additional Information== | ||
PXA belongs to a specific DNA methylation class characterized by BRAF pathway activation (usually BRAF p.V600E) and near‑universal CDKN2A/B homozygous deletion, and is typically IDH1/2‑ and H3‑wildtype; methylation profiling is increasingly used to confirm this integrated molecular diagnosis and to distinguish PXA from pilocytic astrocytoma and epithelioid glioblastoma<ref>{{Cite journal|last=Dampier|first=Christopher H.|last2=Shah|first2=Niharika|last3=Galbraith|first3=Kristyn|last4=Ebrahimi|first4=Azadeh|last5=Neto|first5=Osorio Lopes Abath|last6=Abdullaev|first6=Zied|last7=Alexandrescu|first7=Sanda|last8=Andreiuolo|first8=Felipe|last9=Armstrong|first9=Terri|date=2025|title=Molecular, histologic, and clinical characterization of methylation class pleomorphic xanthoastrocytoma: An analysis of 469 tumors|url=https://pubmed.ncbi.nlm.nih.gov/40735274|journal=Neuro-Oncology Advances|volume=7|issue=1|pages=vdaf089|doi=10.1093/noajnl/vdaf089|issn=2632-2498|pmc=12305539|pmid=40735274}}</ref>. | |||
==Links== | ==Links== | ||
[https://www.cancer.gov/rare-brain-spine-tumor/tumors/pleomorphic-xanthroastrocytoma Pleomorphic Xanthoastrocytoma (PXA) and Other BRAF-Altered Tumors: Diagnosis and Treatment - NCI] | [https://www.cancer.gov/rare-brain-spine-tumor/tumors/pleomorphic-xanthroastrocytoma Pleomorphic Xanthoastrocytoma (PXA) and Other BRAF-Altered Tumors: Diagnosis and Treatment - NCI] | ||