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<meta name="keywords" content="C0334586, neoplastic process, pleomorphic xantho-astrocytoma, pleomorphic xanthoastrocytoma, pxa, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="Pleomorphic xanthoastrocytomas (PXA) are rare low-grade astrocytomas (WHO Grade II) typically found in the temporal lobe and classically presenting with epilepsy. PXA is an astrocytic neoplasm that most often presents in children or young adults but can also occur in adults. The diagnosis of anaplastic PXA is made based upon tumor histopathologic characteristics and requires increased proliferative activity (mitotic index at least 5 mitoses/10 HPF), which is associated with worse overall survival. In general, anaplastic PXAs acquire features of a more aggressive astrocytic neoplasm that can include increased proliferation, necrosis, microvascular proliferation, loss of pericellular reticulin, and increased infiltrative growth." /><meta name="robots" content="index,nofollow,noarchive" />
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<!--
UID=137786
ConceptID=C0334586
-->
<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Pleomorphic xanthoastrocytoma<span class="h1sub">(PXA)</span></div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>137786</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information."><span class="highlight" style="background-color:">C0334586</span></a></dd><dt><span class="dotprefix"></span></dt><dd>Neoplastic Process</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonym:</td>
<td>PXA</td></tr>
<tr><td><span class="bold">SNOMED CT: </span></td>
<td>Pleomorphic xanthoastrocytoma (78838008); Pleomorphic xanthoastrocytoma (189924002)</td></tr>
<tr><td>Modes of inheritance:</td>
<td>
<div class="divPopper rprt" id="moi_988794"><div><strong>Not genetically inherited</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>988794</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier assigned by MedGen (starting with CN) for terms&#10;that cannot be identified in NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">CN307044</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding</dd></dl></div></div></div>
<div class="spaceAbove">Source: Orphanet</div>
<div class="spaceAbove">clinical entity without genetic inheritance.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/988794">This record</a></div></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#moi_988794" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Not genetically inherited</a><span> (Orphanet)</span></div></td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td>HPO:</td>
<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0033682">HP:0033682</a></td></tr>
<tr><td>Monarch Initiative:</td>
<td><a href="https://monarchinitiative.org/disease/MONDO:0016690" target="_blank">MONDO:0016690</a></td></tr>
<tr><td>Orphanet:</td>
<td><a target="_blank" title="Orphanet: The portal for rare diseases and orphan drugs" href="http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&amp;Expert=251607">ORPHA251607</a></td></tr></tbody></table></div><div class="rprt-body jig-ncbiinpagenav" data-jigconfig="smoothScroll: false, gotoTopLink: true, gotoTopLinkText: '', gotoTopLinkAttrs: {'title': 'Go to the top of the page'},allHeadingLevels: ['h1'], topOfPageTOC: true, tocId: 'my-toc'"><div id="rprt-tabs-1" class="rprt-tab"><div id="tb-termsProp-1"><div class="leftCol mgCol"><div>
<div class="portlet mgSection" id="ID_100">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">Pleomorphic xanthoastrocytomas (PXA) are rare low-grade astrocytomas (WHO Grade II) typically found in the temporal lobe and classically presenting with epilepsy. PXA is an astrocytic neoplasm that most often presents in children or young adults but can also occur in adults. The diagnosis of anaplastic PXA is made based upon tumor histopathologic characteristics and requires increased proliferative activity (mitotic index at least 5 mitoses/10 HPF), which is associated with worse overall survival. In general, anaplastic PXAs acquire features of a more aggressive astrocytic neoplasm that can include increased proliferation, necrosis, microvascular proliferation, loss of pericellular reticulin, and increased infiltrative growth. [from <a title="Human Phenotype Ontology" href="http://www.human-phenotype-ontology.org" class="defSource" target="_blank">HPO</a>]</div>
</div>
<div class="portlet mgSection" id="ID_118">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li><li><a href="#tabORDO">Orphanet</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test,  </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test,  </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM,  </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>,  </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar  </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C0334586[DISCUI]&amp;test_type=Clinical" ref="ncbi_uid=137786">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet unavailable" title="OMIM">O</span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&amp;from_uid=137786" ref="ncbi_uid=137786">V</a></span></span><span class="TLline">Pleomorphic xanthoastrocytoma</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/474891" ref="tree=MeSH" title="MedGen record for Congenital Systemic Disorder">Congenital Systemic Disorder</a></span><ul><li><span class="TLline"><a href="/medgen/105425" ref="tree=MeSH" title="MedGen record for Abnormality of the nervous system">Abnormality of the nervous system</a></span><ul><li><span class="TLline"><a href="/medgen/868416" ref="tree=MeSH" title="MedGen record for Abnormal nervous system morphology">Abnormal nervous system morphology</a></span><ul><li><span class="TLline"><a href="/medgen/867607" ref="tree=MeSH" title="MedGen record for Abnormal glial cell morphology">Abnormal glial cell morphology</a></span><ul><li><span class="TLline"><a href="/medgen/867605" ref="tree=MeSH" title="MedGen record for Abnormal astrocyte morphology">Abnormal astrocyte morphology</a></span><ul><li><span class="TLline"><a href="/medgen/438" ref="tree=MeSH" title="MedGen record for Astrocytoma">Astrocytoma</a></span><ul><li><span class="matched_ds">Pleomorphic xanthoastrocytoma</span><ul><li><span class="TLline"><a href="/medgen/859239" ref="tree=MeSH" title="MedGen record for Childhood Pleomorphic Xanthoastrocytoma">Childhood Pleomorphic Xanthoastrocytoma</a></span><ul><li><span class="TLline"><a href="/medgen/857338" ref="tree=MeSH" title="MedGen record for Recurrent Childhood Pleomorphic Xanthoastrocytoma">Recurrent Childhood Pleomorphic Xanthoastrocytoma</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/1660183" ref="tree=MeSH" title="MedGen record for Supratentorial Pleomorphic Xanthoastrocytoma">Supratentorial Pleomorphic Xanthoastrocytoma</a></span></li><li><span class="TLline"><a href="/medgen/1650926" ref="tree=MeSH" title="MedGen record for Temporal Lobe Pleomorphic Xanthoastrocytoma">Temporal Lobe Pleomorphic Xanthoastrocytoma</a></span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div><div id="tabORDO">Follow <a target="_blank" href="http://www.orpha.net/consor/cgi-bin/Disease_Classif.php?lng=EN&amp;data_id=156&amp;PatId=19658&amp;search=Disease_Classif_Simple&amp;new=1" class="ital bold">this link</a> to review classifications for <span class="ital">Pleomorphic xanthoastrocytoma</span> in Orphanet.</div></div></div>
</div>
<div class="portlet mgSection" id="ID_112">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Conditions_with_this_feature">Conditions with this feature</h1><a sid="112" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln clinfeat">
<div class="divPopper rprt" id="rdis_1748029"><div><strong>Mismatch repair cancer syndrome 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1748029</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C5399763</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Lynch syndrome is characterized by an increased risk for colorectal cancer (CRC) and cancers of the endometrium, ovary, stomach, small bowel, urinary tract, biliary tract, brain (usually glioblastoma), skin (sebaceous adenomas, sebaceous carcinomas, and keratoacanthomas), pancreas, and prostate. Cancer risks and age of onset vary depending on the associated gene. Several other cancer types have been reported to occur in individuals with Lynch syndrome (e.g., breast, sarcomas, adrenocortical carcinoma). However, the data are not sufficient to demonstrate that the risk of developing these cancers is increased in individuals with Lynch syndrome.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/1748029">Condition Record</a></div></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1748029" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Mismatch repair cancer syndrome 1</a></div></div>
</div>
<div class="portlet mgSection" id="ID_105">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
<div class="nl"><a target="_blank" href="/pubmed/35945679">pRB immunostaining in the differential diagnosis between pleomorphic xanthoastrocytoma and glioblastoma with giant cells.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Barresi V,
Simbolo M,
Ciaparrone C,
Pedron S,
Mafficini A,
Scarpa A</span><br />
<span class="medgenPMjournal">Histopathology</span>
2022 Nov;81(5):661-669.
Epub 2022 Aug 25
doi: 10.1111/his.14768.
<span class="bold">PMID: </span><a href="/pubmed/35945679" target="_blank">35945679</a><a href="/pmc/articles/PMC9804328" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33970405">Differences in treatment patterns and overall survival between grade II and anaplastic pleomorphic xanthoastrocytomas.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Rodrigues A,
Bhambhvani H,
Medress ZA,
Malhotra S,
Hayden-Gephart M</span><br />
<span class="medgenPMjournal">J Neurooncol</span>
2021 Jun;153(2):321-330.
Epub 2021 May 10
doi: 10.1007/s11060-021-03772-0.
<span class="bold">PMID: </span><a href="/pubmed/33970405" target="_blank">33970405</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28885262">If it is Not a Glioblastoma, Then What is it? A Differential Diagnostic Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Gokden M</span><br />
<span class="medgenPMjournal">Adv Anat Pathol</span>
2017 Nov;24(6):379-391.
doi: 10.1097/PAP.0000000000000170.
<span class="bold">PMID: </span><a href="/pubmed/28885262" target="_blank">28885262</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22pleomorphic%20xanthoastrocytoma%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (15)</a></div></div>
</div>
<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
<div class="portlet mgSection" id="ID_103">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
<div class="nl"><a target="_blank" href="/pubmed/37452934">Benign Glioma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Wu PB,
Filley AC,
Miller ML,
Bruce JN</span><br />
<span class="medgenPMjournal">Adv Exp Med Biol</span>
2023;1405:31-71.
doi: 10.1007/978-3-031-23705-8_2.
<span class="bold">PMID: </span><a href="/pubmed/37452934" target="_blank">37452934</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32348888">Cerebellar Pleomorphic Xanthoastrocytoma with BRAF V600E Mutation.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Kim SH,
Hwang K,
Lee KS,
Choe G,
Kim CY</span><br />
<span class="medgenPMjournal">World Neurosurg</span>
2020 Jul;139:577-581.
Epub 2020 Apr 26
doi: 10.1016/j.wneu.2020.04.113.
<span class="bold">PMID: </span><a href="/pubmed/32348888" target="_blank">32348888</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30187121">Distribution of EGFR amplification, combined chromosome 7 gain and chromosome 10 loss, and TERT promoter mutation in brain tumors and their potential for the reclassification of IDHwt astrocytoma to glioblastoma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Stichel D,
Ebrahimi A,
Reuss D,
Schrimpf D,
Ono T,
Shirahata M,
Reifenberger G,
Weller M,
Hänggi D,
Wick W,
Herold-Mende C,
Westphal M,
Brandner S,
Pfister SM,
Capper D,
Sahm F,
von Deimling A</span><br />
<span class="medgenPMjournal">Acta Neuropathol</span>
2018 Nov;136(5):793-803.
Epub 2018 Sep 5
doi: 10.1007/s00401-018-1905-0.
<span class="bold">PMID: </span><a href="/pubmed/30187121" target="_blank">30187121</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28815663">WHO 2016 Classification of gliomas.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Wesseling P,
Capper D</span><br />
<span class="medgenPMjournal">Neuropathol Appl Neurobiol</span>
2018 Feb;44(2):139-150.
doi: 10.1111/nan.12432.
<span class="bold">PMID: </span><a href="/pubmed/28815663" target="_blank">28815663</a></div>
<div class="nl"><a target="_blank" href="/pubmed/8293185">The new WHO classification of brain tumours.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Kleihues P,
Burger PC,
Scheithauer BW</span><br />
<span class="medgenPMjournal">Brain Pathol</span>
1993 Jul;3(3):255-68.
doi: 10.1111/j.1750-3639.1993.tb00752.x.
<span class="bold">PMID: </span><a href="/pubmed/8293185" target="_blank">8293185</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pleomorphic%20xanthoastrocytoma%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (137)</a></div><h3 class="subhead">Diagnosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/37452934">Benign Glioma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Wu PB,
Filley AC,
Miller ML,
Bruce JN</span><br />
<span class="medgenPMjournal">Adv Exp Med Biol</span>
2023;1405:31-71.
doi: 10.1007/978-3-031-23705-8_2.
<span class="bold">PMID: </span><a href="/pubmed/37452934" target="_blank">37452934</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28815663">WHO 2016 Classification of gliomas.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Wesseling P,
Capper D</span><br />
<span class="medgenPMjournal">Neuropathol Appl Neurobiol</span>
2018 Feb;44(2):139-150.
doi: 10.1111/nan.12432.
<span class="bold">PMID: </span><a href="/pubmed/28815663" target="_blank">28815663</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26948368">Rare glial tumors.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Soffietti R,
Rudà R,
Reardon D</span><br />
<span class="medgenPMjournal">Handb Clin Neurol</span>
2016;134:399-415.
doi: 10.1016/B978-0-12-802997-8.00024-4.
<span class="bold">PMID: </span><a href="/pubmed/26948368" target="_blank">26948368</a></div>
<div class="nl"><a target="_blank" href="/pubmed/25268071">BRAF alterations in brain tumours: molecular pathology and therapeutic opportunities.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Berghoff AS,
Preusser M</span><br />
<span class="medgenPMjournal">Curr Opin Neurol</span>
2014 Dec;27(6):689-96.
doi: 10.1097/WCO.0000000000000146.
<span class="bold">PMID: </span><a href="/pubmed/25268071" target="_blank">25268071</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1414823">Pleomorphic xanthoastrocytoma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Yoshino MT,
Lucio R</span><br />
<span class="medgenPMjournal">AJNR Am J Neuroradiol</span>
1992 Sep-Oct;13(5):1330-2.
<span class="bold">PMID: </span><a href="/pubmed/1414823" target="_blank">1414823</a><a href="/pmc/articles/PMC8335242" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pleomorphic%20xanthoastrocytoma%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (281)</a></div><h3 class="subhead">Therapy</h3>
<div class="nl"><a target="_blank" href="/pubmed/37004712">Recombinant polio-rhinovirus immunotherapy for recurrent paediatric high-grade glioma: a phase 1b trial.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Thompson EM,
Landi D,
Brown MC,
Friedman HS,
McLendon R,
Herndon JE 2nd,
Buckley E,
Bolognesi DP,
Lipp E,
Schroeder K,
Becher OJ,
Friedman AH,
McKay Z,
Walter A,
Threatt S,
Jaggers D,
Desjardins A,
Gromeier M,
Bigner DD,
Ashley DM</span><br />
<span class="medgenPMjournal">Lancet Child Adolesc Health</span>
2023 Jul;7(7):471-478.
Epub 2023 Mar 30
doi: 10.1016/S2352-4642(23)00031-7.
<span class="bold">PMID: </span><a href="/pubmed/37004712" target="_blank">37004712</a><a href="/pmc/articles/PMC11104482" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34848827">Sporadic and Lynch syndrome-associated mismatch repair-deficient brain tumors.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Kim H,
Lim KY,
Park JW,
Kang J,
Won JK,
Lee K,
Shim Y,
Park CK,
Kim SK,
Choi SH,
Kim TM,
Yun H,
Park SH</span><br />
<span class="medgenPMjournal">Lab Invest</span>
2022 Feb;102(2):160-171.
Epub 2021 Nov 30
doi: 10.1038/s41374-021-00694-3.
<span class="bold">PMID: </span><a href="/pubmed/34848827" target="_blank">34848827</a><a href="/pmc/articles/PMC8784316" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30835007">Evidence-Based Practice: Temozolomide Beyond Glioblastoma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Chua J,
Nafziger E,
Leung D</span><br />
<span class="medgenPMjournal">Curr Oncol Rep</span>
2019 Mar 5;21(4):30.
doi: 10.1007/s11912-019-0783-5.
<span class="bold">PMID: </span><a href="/pubmed/30835007" target="_blank">30835007</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28984141">Dabrafenib and trametinib in BRAFV600E mutated glioma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Brown NF,
Carter T,
Kitchen N,
Mulholland P</span><br />
<span class="medgenPMjournal">CNS Oncol</span>
2017 Oct;6(4):291-296.
Epub 2017 Oct 6
doi: 10.2217/cns-2017-0006.
<span class="bold">PMID: </span><a href="/pubmed/28984141" target="_blank">28984141</a><a href="/pmc/articles/PMC6004887" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26287849">Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 Mutations.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Hyman DM,
Puzanov I,
Subbiah V,
Faris JE,
Chau I,
Blay JY,
Wolf J,
Raje NS,
Diamond EL,
Hollebecque A,
Gervais R,
Elez-Fernandez ME,
Italiano A,
Hofheinz RD,
Hidalgo M,
Chan E,
Schuler M,
Lasserre SF,
Makrutzki M,
Sirzen F,
Veronese ML,
Tabernero J,
Baselga J</span><br />
<span class="medgenPMjournal">N Engl J Med</span>
2015 Aug 20;373(8):726-36.
doi: 10.1056/NEJMoa1502309.
<span class="bold">PMID: </span><a href="/pubmed/26287849" target="_blank">26287849</a><a href="/pmc/articles/PMC4971773" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pleomorphic%20xanthoastrocytoma%22%20AND%20Therapy%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (44)</a></div><h3 class="subhead">Prognosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/37452934">Benign Glioma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Wu PB,
Filley AC,
Miller ML,
Bruce JN</span><br />
<span class="medgenPMjournal">Adv Exp Med Biol</span>
2023;1405:31-71.
doi: 10.1007/978-3-031-23705-8_2.
<span class="bold">PMID: </span><a href="/pubmed/37452934" target="_blank">37452934</a></div>
<div class="nl"><a target="_blank" href="/pubmed/31535562">Pleomorphic xanthoastrocytoma: a brief review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Shaikh N,
Brahmbhatt N,
Kruser TJ,
Kam KL,
Appin CL,
Wadhwani N,
Chandler J,
Kumthekar P,
Lukas RV</span><br />
<span class="medgenPMjournal">CNS Oncol</span>
2019 Nov 1;8(3):CNS39.
Epub 2019 Sep 19
doi: 10.2217/cns-2019-0009.
<span class="bold">PMID: </span><a href="/pubmed/31535562" target="_blank">31535562</a><a href="/pmc/articles/PMC6880293" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30187121">Distribution of EGFR amplification, combined chromosome 7 gain and chromosome 10 loss, and TERT promoter mutation in brain tumors and their potential for the reclassification of IDHwt astrocytoma to glioblastoma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Stichel D,
Ebrahimi A,
Reuss D,
Schrimpf D,
Ono T,
Shirahata M,
Reifenberger G,
Weller M,
Hänggi D,
Wick W,
Herold-Mende C,
Westphal M,
Brandner S,
Pfister SM,
Capper D,
Sahm F,
von Deimling A</span><br />
<span class="medgenPMjournal">Acta Neuropathol</span>
2018 Nov;136(5):793-803.
Epub 2018 Sep 5
doi: 10.1007/s00401-018-1905-0.
<span class="bold">PMID: </span><a href="/pubmed/30187121" target="_blank">30187121</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26948368">Rare glial tumors.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Soffietti R,
Rudà R,
Reardon D</span><br />
<span class="medgenPMjournal">Handb Clin Neurol</span>
2016;134:399-415.
doi: 10.1016/B978-0-12-802997-8.00024-4.
<span class="bold">PMID: </span><a href="/pubmed/26948368" target="_blank">26948368</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1414823">Pleomorphic xanthoastrocytoma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Yoshino MT,
Lucio R</span><br />
<span class="medgenPMjournal">AJNR Am J Neuroradiol</span>
1992 Sep-Oct;13(5):1330-2.
<span class="bold">PMID: </span><a href="/pubmed/1414823" target="_blank">1414823</a><a href="/pmc/articles/PMC8335242" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pleomorphic%20xanthoastrocytoma%22%20AND%20Prognosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (207)</a></div><h3 class="subhead">Clinical prediction guides</h3>
<div class="nl"><a target="_blank" href="/pubmed/31535562">Pleomorphic xanthoastrocytoma: a brief review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Shaikh N,
Brahmbhatt N,
Kruser TJ,
Kam KL,
Appin CL,
Wadhwani N,
Chandler J,
Kumthekar P,
Lukas RV</span><br />
<span class="medgenPMjournal">CNS Oncol</span>
2019 Nov 1;8(3):CNS39.
Epub 2019 Sep 19
doi: 10.2217/cns-2019-0009.
<span class="bold">PMID: </span><a href="/pubmed/31535562" target="_blank">31535562</a><a href="/pmc/articles/PMC6880293" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30187121">Distribution of EGFR amplification, combined chromosome 7 gain and chromosome 10 loss, and TERT promoter mutation in brain tumors and their potential for the reclassification of IDHwt astrocytoma to glioblastoma.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Stichel D,
Ebrahimi A,
Reuss D,
Schrimpf D,
Ono T,
Shirahata M,
Reifenberger G,
Weller M,
Hänggi D,
Wick W,
Herold-Mende C,
Westphal M,
Brandner S,
Pfister SM,
Capper D,
Sahm F,
von Deimling A</span><br />
<span class="medgenPMjournal">Acta Neuropathol</span>
2018 Nov;136(5):793-803.
Epub 2018 Sep 5
doi: 10.1007/s00401-018-1905-0.
<span class="bold">PMID: </span><a href="/pubmed/30187121" target="_blank">30187121</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26287849">Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 Mutations.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Hyman DM,
Puzanov I,
Subbiah V,
Faris JE,
Chau I,
Blay JY,
Wolf J,
Raje NS,
Diamond EL,
Hollebecque A,
Gervais R,
Elez-Fernandez ME,
Italiano A,
Hofheinz RD,
Hidalgo M,
Chan E,
Schuler M,
Lasserre SF,
Makrutzki M,
Sirzen F,
Veronese ML,
Tabernero J,
Baselga J</span><br />
<span class="medgenPMjournal">N Engl J Med</span>
2015 Aug 20;373(8):726-36.
doi: 10.1056/NEJMoa1502309.
<span class="bold">PMID: </span><a href="/pubmed/26287849" target="_blank">26287849</a><a href="/pmc/articles/PMC4971773" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/24445767">Molecular genetics of gliomas.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Appin CL,
Brat DJ</span><br />
<span class="medgenPMjournal">Cancer J</span>
2014 Jan-Feb;20(1):66-72.
doi: 10.1097/PPO.0000000000000020.
<span class="bold">PMID: </span><a href="/pubmed/24445767" target="_blank">24445767</a></div>
<div class="nl"><a target="_blank" href="/pubmed/17537486">Cerebellar pleomorphic xanthoastrocytoma: case report and literature review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Hamlat A,
Le Strat A,
Guegan Y,
Ben-Hassel M,
Saikali S</span><br />
<span class="medgenPMjournal">Surg Neurol</span>
2007 Jul;68(1):89-94; discussion 94-5.
Epub 2007 May 29
doi: 10.1016/j.surneu.2006.08.064.
<span class="bold">PMID: </span><a href="/pubmed/17537486" target="_blank">17537486</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pleomorphic%20xanthoastrocytoma%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (108)</a></div></div>
</div>
<div class="portlet mgSection" id="ID_104">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_systematic_reviews">Recent systematic reviews</h1><a sid="104" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">
<div class="nl"><a target="_blank" href="/pubmed/37743598">The Significance of BRAF Mutation in the Epithelioid Glioblastoma Subtype: A Systematic Literature Review and a Case Report with a Unique Intraventricular Topography.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Prieto R,
Barrios L,
Ebrat-Mancilla E,
Martín P,
Tejerina E</span><br />
<span class="medgenPMjournal">Int J Surg Pathol</span>
2024 Jun;32(4):649-666.
Epub 2023 Sep 24
doi: 10.1177/10668969231195026.
<span class="bold">PMID: </span><a href="/pubmed/37743598" target="_blank">37743598</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Andrews LJ,
Thornton ZA,
Saincher SS,
Yao IY,
Dawson S,
McGuinness LA,
Jones HE,
Jefferies S,
Short SC,
Cheng HY,
McAleenan A,
Higgins JPT,
Kurian KM</span><br />
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2022 Apr 1;24(4):528-540.
doi: 10.1093/neuonc/noab247.
<span class="bold">PMID: </span><a href="/pubmed/34718782" target="_blank">34718782</a><a href="/pmc/articles/PMC8972326" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33160715">Cerebellar pleomorphic xanthoastrocytoma in the setting of neurofibromatosis type-I: Does it portend a different prognosis? A case report and systematic review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Mathkour M,
Banerjee S,
Werner C,
Hanna J,
Abou-Al-Shaar H,
Dindial R,
Scullen T,
Boehm L,
Tubbs RS,
Ware ML</span><br />
<span class="medgenPMjournal">Clin Neurol Neurosurg</span>
2021 Jan;200:106346.
Epub 2020 Nov 2
doi: 10.1016/j.clineuro.2020.106346.
<span class="bold">PMID: </span><a href="/pubmed/33160715" target="_blank">33160715</a></div>
<div class="nl"><a target="_blank" href="/pubmed/31147232">Clinical relevance of BRAF status in glial and glioneuronal tumors: A systematic review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Sugiura Y,
Nagaishi M</span><br />
<span class="medgenPMjournal">J Clin Neurosci</span>
2019 Aug;66:196-201.
Epub 2019 May 27
doi: 10.1016/j.jocn.2019.05.014.
<span class="bold">PMID: </span><a href="/pubmed/31147232" target="_blank">31147232</a></div>
<div class="nl"><a target="_blank" href="/pubmed/29803334">Grade II Pleomorphic Xanthoastrocytoma; a meta-analysis of data from previously reported 167 cases.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Mallick S,
Benson R,
Melgandi W,
Giridhar P,
Rath GK</span><br />
<span class="medgenPMjournal">J Clin Neurosci</span>
2018 Aug;54:57-62.
Epub 2018 May 24
doi: 10.1016/j.jocn.2018.05.003.
<span class="bold">PMID: </span><a href="/pubmed/29803334" target="_blank">29803334</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Pleomorphic%20xanthoastrocytoma%22%20AND%20systematic%5Bsb%5D%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (5)</a></div></div>
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