CNS5:Pleomorphic xanthoastrocytoma - Revision history

Richard.Glen: Section title fix

2026-04-21T18:49:51Z

Section title fix

← Older revision		Revision as of 13:49, 21 April 2026
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	== Related Terminology ~~- Pleomorphic xanthoastrocytoma (PXA)~~ ==		== Related Terminology ==

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Laveniya.Satgunaseelan: final edits - gene italicisation mainly

2026-04-02T11:43:49Z

final edits - gene italicisation mainly

Show changes

Richard.Glen at 01:25, 26 February 2026

2026-02-26T01:25:21Z

← Older revision		Revision as of 20:25, 25 February 2026
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	[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]
	==References==		==References==
			<references />

	==Notes==		==Notes==
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	[[Category:DISEASE]]		[[Category:DISEASE]]
	[[Category:Diseases P]]		[[Category:Diseases P]]
	~~<references />~~

Wkhan at 08:04, 8 February 2026

2026-02-08T08:04:05Z

← Older revision		Revision as of 03:04, 8 February 2026
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	==Related Terminology==		== Related Terminology - Pleomorphic xanthoastrocytoma (PXA) ==

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	==Gene Rearrangements==		==Gene Rearrangements ==

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Wkhan at 08:01, 8 February 2026

2026-02-08T08:01:16Z

← Older revision		Revision as of 03:01, 8 February 2026
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	==Genes and Main Pathways Involved==		==Genes and Main Pathways Involved==
	~~Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Please include references throughout the table. Do not delete the table.)''</span>~~
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Wkhan at 07:45, 8 February 2026

2026-02-08T07:45:09Z

← Older revision		Revision as of 02:45, 8 February 2026
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	[[CNS5:Table_of_Contents\|Central Nervous System Tumours (WHO Classification, 5th ed.)]]		[[CNS5:Table_of_Contents\|Central Nervous System Tumours (WHO Classification, 5th ed.)]]

	<span style="color:#0070C0">(''General Instructions – The focus of these pages is the clinically significant genetic alterations in each disease type. This is based on up-to-date knowledge from multiple resources such as PubMed and the WHO classification books. The CCGA is meant to be a supplemental resource to the WHO classification books; the CCGA captures in a continually updated wiki-stye manner the current genetics/genomics knowledge of each disease, which evolves more rapidly than books can be revised and published. If the same disease is described in multiple WHO classification books, the genetics-related information for that disease will be consolidated into a single main page that has this template (other pages would only contain a link to this main page). Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ <u>HGVS-based nomenclature for variants</u>], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples); to add (or move) a row or column in a table, click nearby within the table and select the > symbol that appears. Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see'' </span><u>''[[Author_Instructions]]''</u><span style="color:#0070C0"> ''and [[Frequently Asked Questions (FAQs)\|<u>FAQs</u>]] as well as contact your [[Leadership\|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>].)''</span>		<span style="color:#0070C0">(''General Instructions – The focus of these pages is the clinically significant genetic alterations in each disease type. This is based on up-to-date knowledge from multiple resources such as PubMed and the WHO classification books. The CCGA is meant to be a supplemental resource to the WHO classification books; the CCGA captures in a continually updated wiki-stye manner the current genetics/genomics knowledge of each disease, which evolves more rapidly than books can be revised and published. If the same disease is described in multiple WHO classification books, the genetics-related information for that disease will be consolidated into a single main page that has this template (other pages would only contain a link to this main page).'' </span>
	==Primary Author(s)*==		==Primary Author(s)*==
	Wahab A. Khan, PhD, FACMG, Dartmouth Health <span style="color:#0070C0"> </span>		Wahab A. Khan, PhD, FACMG, Dartmouth Health <span style="color:#0070C0"> </span>
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	==Gene Rearrangements==		==Gene Rearrangements==
	Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Details on clinical significance such as prognosis and other important information can be provided in the notes section. Please include references throughout the table. Do not delete the table.'')</span>
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	==Individual Region Genomic Gain/Loss/LOH==		==Individual Region Genomic Gain/Loss/LOH==
	Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Includes aberrations not involving gene rearrangements. Details on clinical significance such as prognosis and other important information can be provided in the notes section. Can refer to CGC workgroup tables as linked on the homepage if applicable. Please include references throughout the table. Do not delete the table.'') </span>
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	==Characteristic Chromosomal or Other Global Mutational Patterns==		==Characteristic Chromosomal or Other Global Mutational Patterns==
	Put your text here and fill in the table <span style="color:#0070C0">(I''nstructions: Included in this category are alterations such as hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis; microsatellite instability; homologous recombination deficiency; mutational signature pattern; etc. Details on clinical significance such as prognosis and other important information can be provided in the notes section. Please include references throughout the table. Do not delete the table.'')</span>
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	==Gene Mutations (SNV/INDEL)==		==Gene Mutations (SNV/INDEL)==
	~~Put your text here and fill in the table~~ <span style="color:#0070C0">(''~~Instructions:~~ This table is not meant to be an exhaustive list; please include only genes/alterations that are recurrent or common as well either disease defining and/or clinically significant. If a gene has multiple mechanisms depending on the type or site of the alteration, add multiple entries in the table. For clinical significance, denote associations with FDA-approved therapy (not an extensive list of applicable drugs) and NCCN or other national guidelines if applicable; Can also refer to CGC workgroup tables as linked on the homepage if applicable as well as any high impact papers or reviews of gene mutations in this entity. Details on clinical significance such as prognosis and other important information such as concomitant and mutually exclusive mutations can be provided in the notes section. Please include references throughout the table. Do not delete the table.'') </span>		<span style="color:#0070C0">''This table is not meant to be an exhaustive list''</span>
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	[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]
	==References==		==References==
	(use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking where you want to insert the reference, selecting the “Cite” icon at the top of the wiki page, and using the “Automatic” tab option to search by PMID to select the reference to insert. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference. To insert the same reference again later in the page, select the “Cite” icon and “Re-use” to find the reference; DO NOT insert the same reference twice using the “Automatic” tab as it will be treated as two separate references. The reference list in this section will be automatically generated and sorted''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">)</span>
	==Notes==		==Notes==
	<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the [[Leadership\|''<u>Associate Editor</u>'']] or other CCGA representative. When pages have a major update, the new author will be acknowledged at the beginning of the page, and those who contributed previously will be acknowledged below as a prior author.		Khan WA: “Pleomorphic xanthoastrocytoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/CNS5:Pleomorphic xanthoastrocytoma</nowiki>.

	~~Prior Author(s):~~
	~~<nowiki>*</nowiki>''Citation of this Page''~~: “Pleomorphic xanthoastrocytoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/CNS5:Pleomorphic xanthoastrocytoma</nowiki>.
	[[Category:CNS5]]		[[Category:CNS5]]
	[[Category:DISEASE]]		[[Category:DISEASE]]
	[[Category:Diseases P]]		[[Category:Diseases P]]
	<references />		<references />

Wkhan: /* Additional Information */

2026-02-08T07:37:43Z

Additional Information

← Older revision		Revision as of 02:37, 8 February 2026
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	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==
	~~Put your text here~~		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]

Wkhan at 07:34, 8 February 2026

2026-02-08T07:34:28Z

← Older revision		Revision as of 02:34, 8 February 2026
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	[[CNS5:Table_of_Contents\|Central Nervous System Tumours (WHO Classification, 5th ed.)]]		[[CNS5:Table_of_Contents\|Central Nervous System Tumours (WHO Classification, 5th ed.)]]

	~~{{Under Construction}}~~

	<span style="color:#0070C0">(''General Instructions – The focus of these pages is the clinically significant genetic alterations in each disease type. This is based on up-to-date knowledge from multiple resources such as PubMed and the WHO classification books. The CCGA is meant to be a supplemental resource to the WHO classification books; the CCGA captures in a continually updated wiki-stye manner the current genetics/genomics knowledge of each disease, which evolves more rapidly than books can be revised and published. If the same disease is described in multiple WHO classification books, the genetics-related information for that disease will be consolidated into a single main page that has this template (other pages would only contain a link to this main page). Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ <u>HGVS-based nomenclature for variants</u>], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples); to add (or move) a row or column in a table, click nearby within the table and select the > symbol that appears. Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see'' </span><u>''[[Author_Instructions]]''</u><span style="color:#0070C0"> ''and [[Frequently Asked Questions (FAQs)\|<u>FAQs</u>]] as well as contact your [[Leadership\|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>].)''</span>		<span style="color:#0070C0">(''General Instructions – The focus of these pages is the clinically significant genetic alterations in each disease type. This is based on up-to-date knowledge from multiple resources such as PubMed and the WHO classification books. The CCGA is meant to be a supplemental resource to the WHO classification books; the CCGA captures in a continually updated wiki-stye manner the current genetics/genomics knowledge of each disease, which evolves more rapidly than books can be revised and published. If the same disease is described in multiple WHO classification books, the genetics-related information for that disease will be consolidated into a single main page that has this template (other pages would only contain a link to this main page). Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ <u>HGVS-based nomenclature for variants</u>], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples); to add (or move) a row or column in a table, click nearby within the table and select the > symbol that appears. Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see'' </span><u>''[[Author_Instructions]]''</u><span style="color:#0070C0"> ''and [[Frequently Asked Questions (FAQs)\|<u>FAQs</u>]] as well as contact your [[Leadership\|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>].)''</span>

Wkhan: /* Genes and Main Pathways Involved */

2026-02-08T07:29:26Z

Genes and Main Pathways Involved

← Older revision		Revision as of 02:29, 8 February 2026
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	\|Unregulated cell division		\|Unregulated cell division
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	\|~~''KMT2C'' and ''ARID1A''; Inactivating~~ mutations		\|TERT promoter mutations or amplification
	\| ~~Histone modification, chromatin remodeling~~		\|TERT – Telomere maintenance pathway
	~~\|Abnormal gene expression program~~		\|Activate telomerase and support immortalization via the telomere maintenance pathway
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	==Genetic Diagnostic Testing Methods==		==Genetic Diagnostic Testing Methods==

Wkhan: /* Genes and Main Pathways Involved */

2026-02-08T07:25:41Z

Genes and Main Pathways Involved

← Older revision		Revision as of 02:25, 8 February 2026
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	!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome		!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome
	\|-		\|-
	\|~~<span class="blue-text">EXAMPLE:</span>~~ ''BRAF'' and ''MAP2K1''; Activating mutations		\|''BRAF'' and ''MAP2K1''; Activating mutations
	\|~~<span class="blue-text">EXAMPLE:</span>~~ MAPK signaling		\|MAPK signaling
	\|~~<span class="blue-text">EXAMPLE:</span>~~ Increased cell growth and proliferation		\|Increased cell growth and proliferation
	\|-		\|-
	\|~~<span class="blue-text">EXAMPLE:</span>~~ ''CDKN2A''; Inactivating mutations		\|''CDKN2A''; Inactivating mutations
	\|~~<span class="blue-text">EXAMPLE:</span>~~ Cell cycle regulation		\|Cell cycle regulation
	\|~~<span class="blue-text">EXAMPLE:</span>~~ Unregulated cell division		\|Unregulated cell division
	\|-		\|-
	\|~~<span class="blue-text">EXAMPLE:</span>~~ ''KMT2C'' and ''ARID1A''; Inactivating mutations		\|''KMT2C'' and ''ARID1A''; Inactivating mutations
	\|~~<span class="blue-text">EXAMPLE:</span>~~ Histone modification, chromatin remodeling		\| Histone modification, chromatin remodeling
	\|~~<span class="blue-text">EXAMPLE:</span> Abnormal~~ gene expression program		\|Abnormal gene expression program
	\|-		\|-
	\|		\|