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<meta name="DC.Title" content="MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children's Oncology Group ACNS0332 Trial" />
<meta name="DC.Identifier" content="10.3174/ajnr.A6253" />
<meta name="DC.Date" content="2019-11-01" />
<meta name="DC.Publisher" content="American Journal of Neuroradiology" />
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<meta name="DC.AccessRights" content="open-access" />
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<meta name="DC.Description" content="BACKGROUND AND PURPOSE: Supratentorial primitive neuroectodermal tumors and pineoblastomas have traditionally been grouped together for treatment purposes. Molecular profiling of these tumors has revealed a number of distinct entities and has led to the term “CNS-primitive neuroectodermal tumors” being removed from the 2016 World Health Organization classification. The purpose of this study was to describe the MR imaging findings of histologically diagnosed primitive neuroectodermal tumors and pineoblastomas and correlate them with molecular diagnoses and outcomes.

MATERIALS AND METHODS: Histologically diagnosed primitive neuroectodermal tumors and pineoblastomas were enrolled in this Children's Oncology Group Phase III trial, and molecular classification was retrospectively completed using DNA methylation profiling. MR imaging features were systematically studied and correlated with molecular diagnoses and survival.

RESULTS: Of the 85 patients enrolled, 56 met the inclusion criteria, in whom 28 tumors were in pineal and 28 in nonpineal locations. Methylation profiling revealed a variety of diagnoses, including pineoblastomas ( n = 27), high-grade gliomas ( n = 17), embryonal tumors ( n = 7), atypical teratoid/rhabdoid tumors ( n = 3), and ependymomas ( n = 2). Thus, 39% overall and 71% of nonpineal tumor diagnoses were discrepant with histopathology. Tumor location, size, margins, and edema were predictors of embryonal-versus-nonembryonal tumors. Larger size and ill-defined margins correlated with poor event-free survival, while metastatic disease by MR imaging did not.

CONCLUSIONS: In nonpineal locations, only a minority of histologically diagnosed primitive neuroectodermal tumors are embryonal tumors; therefore, high-grade glioma or ependymoma should be high on the radiographic differential. An understanding of molecularly defined tumor entities and their relative frequencies and locations will help the radiologist make more accurate predictions of the tumor types.

ATRT
:   atypical teratoid/rhabdoid tumor
EP
:   ependymoma
ET
:   embryonal tumor
ETMR
:   embryonal tumor with multilayered rosettes
GBM
:   glioblastoma multiforme
HGG
:   high-grade glioma
HGNET
:   high-grade neuroepithelial tumor
MB
:   medulloblastoma
NOS
:   not otherwise specified
PBL
:   pineoblastoma
PNET
:   primitive neuroectodermal tumor" />
<meta name="DC.Contributor" content="A. Jaju" />
<meta name="DC.Contributor" content="E.I. Hwang" />
<meta name="DC.Contributor" content="M. Kool" />
<meta name="DC.Contributor" content="D. Capper" />
<meta name="DC.Contributor" content="L. Chavez" />
<meta name="DC.Contributor" content="S. Brabetz" />
<meta name="DC.Contributor" content="C. Billups" />
<meta name="DC.Contributor" content="Y. Li" />
<meta name="DC.Contributor" content="M. Fouladi" />
<meta name="DC.Contributor" content="R.J. Packer" />
<meta name="DC.Contributor" content="S.M. Pfister" />
<meta name="DC.Contributor" content="J.M. Olson" />
<meta name="DC.Contributor" content="L.A. Heier" />
<meta name="article:published_time" content="2019-11-01" />
<meta name="article:section" content="Pediatrics" />
<meta name="citation_title" content="MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children's Oncology Group ACNS0332 Trial" />
<meta name="citation_abstract" lang="en" content="&lt;h3&gt;BACKGROUND AND PURPOSE:&lt;/h3&gt;
&lt;p&gt;Supratentorial primitive neuroectodermal tumors and pineoblastomas have traditionally been grouped together for treatment purposes. Molecular profiling of these tumors has revealed a number of distinct entities and has led to the term “CNS-primitive neuroectodermal tumors” being removed from the 2016 World Health Organization classification. The purpose of this study was to describe the MR imaging findings of histologically diagnosed primitive neuroectodermal tumors and pineoblastomas and correlate them with molecular diagnoses and outcomes.&lt;/p&gt;&lt;h3&gt;MATERIALS AND METHODS:&lt;/h3&gt;
&lt;p&gt;Histologically diagnosed primitive neuroectodermal tumors and pineoblastomas were enrolled in this Children9s Oncology Group Phase III trial, and molecular classification was retrospectively completed using DNA methylation profiling. MR imaging features were systematically studied and correlated with molecular diagnoses and survival.&lt;/p&gt;&lt;h3&gt;RESULTS:&lt;/h3&gt;
&lt;p&gt;Of the 85 patients enrolled, 56 met the inclusion criteria, in whom 28 tumors were in pineal and 28 in nonpineal locations. Methylation profiling revealed a variety of diagnoses, including pineoblastomas (&lt;i&gt;n&lt;/i&gt; = 27), high-grade gliomas (&lt;i&gt;n&lt;/i&gt; = 17), embryonal tumors (&lt;i&gt;n&lt;/i&gt; = 7), atypical teratoid/rhabdoid tumors (&lt;i&gt;n&lt;/i&gt; = 3), and ependymomas (&lt;i&gt;n&lt;/i&gt; = 2). Thus, 39% overall and 71% of nonpineal tumor diagnoses were discrepant with histopathology. Tumor location, size, margins, and edema were predictors of embryonal-versus-nonembryonal tumors. Larger size and ill-defined margins correlated with poor event-free survival, while metastatic disease by MR imaging did not.&lt;/p&gt;&lt;h3&gt;CONCLUSIONS:&lt;/h3&gt;
&lt;p&gt;In nonpineal locations, only a minority of histologically diagnosed primitive neuroectodermal tumors are embryonal tumors; therefore, high-grade glioma or ependymoma should be high on the radiographic differential. An understanding of molecularly defined tumor entities and their relative frequencies and locations will help the radiologist make more accurate predictions of the tumor types.&lt;/p&gt;" />
<meta name="citation_journal_title" content="American Journal of Neuroradiology" />
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<meta name="citation_publication_date" content="2019/11/01" />
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<meta name="citation_author" content="A. Jaju" />
<meta name="citation_author_institution" content="aFrom the Department of Radiology (A.J.), Ann and Robert H Lurie Children's Hospital of Chicago, Chicago, Illinois" />
<meta name="citation_author_institution" content="bNorthwestern University Feinberg School of Medicine (A.J.), Chicago, Illinois" />
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<meta name="citation_author" content="M. Kool" />
<meta name="citation_author_institution" content="dDepartment of Pediatric Neurooncology (M.K., S.B., S.M.P.), German Cancer Research Center, Heidelberg, Baden-Württemberg, Germany" />
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<meta name="citation_author_institution" content="eDepartment of Pediatric Neuropathology (D.C.), University Hospital Heidelberg, Heidelberg, Baden-Württemberg, Germany" />
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<meta name="twitter:title" content="MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children's Oncology Group ACNS0332 Trial" />
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<meta name="twitter:description" content="BACKGROUND AND PURPOSE: Supratentorial primitive neuroectodermal tumors and pineoblastomas have traditionally been grouped together for treatment purposes. Molecular profiling of these tumors has revealed a number of distinct entities and has led to the term “CNS-primitive neuroectodermal tumors” being removed from the 2016 World Health Organization classification. The purpose of this study was to describe the MR imaging findings of histologically diagnosed primitive neuroectodermal tumors and pineoblastomas and correlate them with molecular diagnoses and outcomes.

MATERIALS AND METHODS: Histologically diagnosed primitive neuroectodermal tumors and pineoblastomas were enrolled in this Children's Oncology Group Phase III trial, and molecular classification was retrospectively completed using DNA methylation profiling. MR imaging features were systematically studied and correlated with molecular diagnoses and survival.

RESULTS: Of the 85 patients enrolled, 56 met the inclusion criteria, in whom 28 tumors were in pineal and 28 in nonpineal locations. Methylation profiling revealed a variety of diagnoses, including pineoblastomas ( n = 27), high-grade gliomas ( n = 17), embryonal tumors ( n = 7), atypical teratoid/rhabdoid tumors ( n = 3), and ependymomas ( n = 2). Thus, 39% overall and 71% of nonpineal tumor diagnoses were discrepant with histopathology. Tumor location, size, margins, and edema were predictors of embryonal-versus-nonembryonal tumors. Larger size and ill-defined margins correlated with poor event-free survival, while metastatic disease by MR imaging did not.

CONCLUSIONS: In nonpineal locations, only a minority of histologically diagnosed primitive neuroectodermal tumors are embryonal tumors; therefore, high-grade glioma or ependymoma should be high on the radiographic differential. An understanding of molecularly defined tumor entities and their relative frequencies and locations will help the radiologist make more accurate predictions of the tumor types.

ATRT
:   atypical teratoid/rhabdoid tumor
EP
:   ependymoma
ET
:   embryonal tumor
ETMR
:   embryonal tumor with multilayered rosettes
GBM
:   glioblastoma multiforme
HGG
:   high-grade glioma
HGNET
:   high-grade neuroepithelial tumor
MB
:   medulloblastoma
NOS
:   not otherwise specified
PBL
:   pineoblastoma
PNET
:   primitive neuroectodermal tumor" />
<meta name="og-title" property="og:title" content="MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children's Oncology Group ACNS0332 Trial" />
<meta name="og-url" property="og:url" content="http://ajnr-stage2.stage.highwire.org/content/40/11/1796" />
<meta name="og-site-name" property="og:site_name" content="American Journal of Neuroradiology" />
<meta name="og-description" property="og:description" content="BACKGROUND AND PURPOSE: Supratentorial primitive neuroectodermal tumors and pineoblastomas have traditionally been grouped together for treatment purposes. Molecular profiling of these tumors has revealed a number of distinct entities and has led to the term “CNS-primitive neuroectodermal tumors” being removed from the 2016 World Health Organization classification. The purpose of this study was to describe the MR imaging findings of histologically diagnosed primitive neuroectodermal tumors and pineoblastomas and correlate them with molecular diagnoses and outcomes.

MATERIALS AND METHODS: Histologically diagnosed primitive neuroectodermal tumors and pineoblastomas were enrolled in this Children's Oncology Group Phase III trial, and molecular classification was retrospectively completed using DNA methylation profiling. MR imaging features were systematically studied and correlated with molecular diagnoses and survival.

RESULTS: Of the 85 patients enrolled, 56 met the inclusion criteria, in whom 28 tumors were in pineal and 28 in nonpineal locations. Methylation profiling revealed a variety of diagnoses, including pineoblastomas ( n = 27), high-grade gliomas ( n = 17), embryonal tumors ( n = 7), atypical teratoid/rhabdoid tumors ( n = 3), and ependymomas ( n = 2). Thus, 39% overall and 71% of nonpineal tumor diagnoses were discrepant with histopathology. Tumor location, size, margins, and edema were predictors of embryonal-versus-nonembryonal tumors. Larger size and ill-defined margins correlated with poor event-free survival, while metastatic disease by MR imaging did not.

CONCLUSIONS: In nonpineal locations, only a minority of histologically diagnosed primitive neuroectodermal tumors are embryonal tumors; therefore, high-grade glioma or ependymoma should be high on the radiographic differential. An understanding of molecularly defined tumor entities and their relative frequencies and locations will help the radiologist make more accurate predictions of the tumor types.

ATRT
:   atypical teratoid/rhabdoid tumor
EP
:   ependymoma
ET
:   embryonal tumor
ETMR
:   embryonal tumor with multilayered rosettes
GBM
:   glioblastoma multiforme
HGG
:   high-grade glioma
HGNET
:   high-grade neuroepithelial tumor
MB
:   medulloblastoma
NOS
:   not otherwise specified
PBL
:   pineoblastoma
PNET
:   primitive neuroectodermal tumor" />
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    <title>MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children's Oncology Group ACNS0332 Trial | American Journal of Neuroradiology</title>  
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    <div class="highwire-article-citation highwire-citation-type-highwire-article node71286--5" data-node-nid="71286" id="top-node-71286--62088192958" data-pisa="ajnr;40/11/1796" data-pisa-master="ajnr;ajnr.A6253" data-apath="/ajnr/40/11/1796.atom" data-hw-author-tooltip-instance="highwire_author_tooltip"><div class="highwire-cite highwire-cite-highwire-article highwire-citation-jcore-article-title-complete clearfix has-author-tooltip highwire-citation-highwire-article-top-a">
  
  
      <div class="highwire-cite-overline"><span  class="highwire-cite-metadata-article-type highwire-cite-metadata">Research Article</span><span class="separator-pipe"></span><span  class="highwire-cite-metadata-art-cat highwire-cite-metadata"><span class="wrapper">Pediatrics</span></span></div>
  
      <div class="highwire-cite-access"><span class="highwire-citation-access"><i class="highwire-access-icon highwire-access-icon-open-access open-access hw-icon-open-access" title="Open Access" aria-hidden="true"></i><span  class="element-invisible highwire-access-icon highwire-access-icon-open-access" style="">Open Access</span></span></div>
  
      <h1 class="highwire-cite-title" id="page-title">MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children's Oncology Group ACNS0332 Trial</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">A. Jaju</span>, <span class="highwire-citation-author" data-delta="1">E.I. Hwang</span>, <span class="highwire-citation-author" data-delta="2">M. Kool</span>, <span class="highwire-citation-author" data-delta="3">D. Capper</span>, <span class="highwire-citation-author" data-delta="4">L. Chavez</span>, <span class="highwire-citation-author" data-delta="5">S. Brabetz</span>, <span class="highwire-citation-author" data-delta="6">C. Billups</span>, <span class="highwire-citation-author" data-delta="7">Y. Li</span>, <span class="highwire-citation-author" data-delta="8">M. Fouladi</span>, <span class="highwire-citation-author" data-delta="9">R.J. Packer</span>, <span class="highwire-citation-author" data-delta="10">S.M. Pfister</span>, <span class="highwire-citation-author" data-delta="11">J.M. Olson</span> and <span class="highwire-citation-author" data-delta="12">L.A. Heier</span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">November 2019,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">40 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(11) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1796-1803; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A6253 </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-71286--62088192958" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">A. Jaju </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Department of Radiology (A.J.), Ann and Robert H Lurie Children's Hospital of Chicago, Chicago, Illinois</div><div class='author-affiliation'><span class='nlm-sup'>b</span>Northwestern University Feinberg School of Medicine (A.J.), Chicago, Illinois</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=A.%2BJaju%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Jaju%20A&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AA.%2BJaju%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0001-5025-3709" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for A. Jaju</a></li></ul></div><div class="author-tooltip-1"><div class="author-tooltip-name">E.I. Hwang </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>c</span>Brain Tumor Institute (E.I.H., R.J.P.), Children's National Health System, Washington, DC</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=E.I.%2BHwang%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Hwang%20E&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AE.I.%2BHwang%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0002-3923-1135" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for E.I. Hwang</a></li></ul></div><div class="author-tooltip-2"><div class="author-tooltip-name">M. Kool </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>d</span>Department of Pediatric Neurooncology (M.K., S.B., S.M.P.), German Cancer Research Center, Heidelberg, Baden-Württemberg, Germany</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=M.%2BKool%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Kool%20M&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AM.%2BKool%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0002-6557-5468" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for M. Kool</a></li></ul></div><div class="author-tooltip-3"><div class="author-tooltip-name">D. Capper </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>e</span>Department of Pediatric Neuropathology (D.C.), University Hospital Heidelberg, Heidelberg, Baden-Württemberg, Germany</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=D.%2BCapper%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Capper%20D&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AD.%2BCapper%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0003-1945-497X" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for D. Capper</a></li></ul></div><div class="author-tooltip-4"><div class="author-tooltip-name">L. Chavez </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>f</span>Department of Medicine (L.C.), University of California San Diego, La Jolla, California</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=L.%2BChavez%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Chavez%20L&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AL.%2BChavez%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0002-8718-8848" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for L. Chavez</a></li></ul></div><div class="author-tooltip-5"><div class="author-tooltip-name">S. Brabetz </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>d</span>Department of Pediatric Neurooncology (M.K., S.B., S.M.P.), German Cancer Research Center, Heidelberg, Baden-Württemberg, Germany</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=S.%2BBrabetz%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Brabetz%20S&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AS.%2BBrabetz%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0002-8553-0814" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for S. Brabetz</a></li></ul></div><div class="author-tooltip-6"><div class="author-tooltip-name">C. Billups </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>g</span>Department of Biostatistics (C.B., Y.L.), St. Jude Children's Research Hospital, Memphis, Tennessee</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=C.%2BBillups%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Billups%20C&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AC.%2BBillups%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0003-2819-4946" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for C. Billups</a></li></ul></div><div class="author-tooltip-7"><div class="author-tooltip-name">Y. Li </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>g</span>Department of Biostatistics (C.B., Y.L.), St. Jude Children's Research Hospital, Memphis, Tennessee</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Y.%2BLi%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Li%20Y&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AY.%2BLi%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0002-7046-4316" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for Y. Li</a></li></ul></div><div class="author-tooltip-8"><div class="author-tooltip-name">M. Fouladi </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>h</span>Brain Tumor Center (M.F.), Cincinnati Children's Hospital, Cincinnati, Ohio</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=M.%2BFouladi%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Fouladi%20M&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AM.%2BFouladi%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0002-5693-035X" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for M. Fouladi</a></li></ul></div><div class="author-tooltip-9"><div class="author-tooltip-name">R.J. Packer </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>c</span>Brain Tumor Institute (E.I.H., R.J.P.), Children's National Health System, Washington, DC</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=R.J.%2BPacker%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Packer%20R&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AR.J.%2BPacker%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0001-9413-7531" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for R.J. Packer</a></li></ul></div><div class="author-tooltip-10"><div class="author-tooltip-name">S.M. Pfister </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>d</span>Department of Pediatric Neurooncology (M.K., S.B., S.M.P.), German Cancer Research Center, Heidelberg, Baden-Württemberg, Germany</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=S.M.%2BPfister%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Pfister%20S&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AS.M.%2BPfister%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0002-5447-5322" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for S.M. 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    <div class="messages highwire-corrections highwire-correction-type-correction"><h3 class="highwire-corrections-title">This article has a correction. Please see:</h3><ul class="highwire-corrections-list"><li class="relation-6350 first last"><a href="/content/41/2/E9" class="" data-icon-position="" data-hide-link-title="0">Erratum - February 01, 2020</a></li></ul></div>  </div>

  
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    <div class="highwire-markup"><div xmlns="http://www.w3.org/1999/xhtml" data-highwire-cite-ref-tooltip-instance="highwire_reflinks_tooltip" class="content-block-markup" xmlns:xhtml="http://www.w3.org/1999/xhtml"><div class="article abstract-view "><span class="highwire-journal-article-marker-start"></span><div class="section abstract" id="abstract-1"><h2>Abstract</h2><div id="sec-1" class="subsection"><p id="p-2"><strong>BACKGROUND AND PURPOSE:</strong> Supratentorial primitive neuroectodermal tumors and pineoblastomas have traditionally been grouped together for treatment purposes. Molecular profiling of these tumors has revealed a number of distinct entities and has led to the term “CNS-primitive neuroectodermal tumors” being removed from the 2016 World Health Organization classification. The purpose of this study was to describe the MR imaging findings of histologically diagnosed primitive neuroectodermal tumors and pineoblastomas and correlate them with molecular diagnoses and outcomes.</p></div><div id="sec-2" class="subsection"><p id="p-3"><strong>MATERIALS AND METHODS:</strong> Histologically diagnosed primitive neuroectodermal tumors and pineoblastomas were enrolled in this Children's Oncology Group Phase III trial, and molecular classification was retrospectively completed using DNA methylation profiling. MR imaging features were systematically studied and correlated with molecular diagnoses and survival.</p></div><div id="sec-3" class="subsection"><p id="p-4"><strong>RESULTS:</strong> Of the 85 patients enrolled, 56 met the inclusion criteria, in whom 28 tumors were in pineal and 28 in nonpineal locations. Methylation profiling revealed a variety of diagnoses, including pineoblastomas (<em>n</em> = 27), high-grade gliomas (<em>n</em> = 17), embryonal tumors (<em>n</em> = 7), atypical teratoid/rhabdoid tumors (<em>n</em> = 3), and ependymomas (<em>n</em> = 2). Thus, 39% overall and 71% of nonpineal tumor diagnoses were discrepant with histopathology. Tumor location, size, margins, and edema were predictors of embryonal-versus-nonembryonal tumors. Larger size and ill-defined margins correlated with poor event-free survival, while metastatic disease by MR imaging did not.</p></div><div id="sec-4" class="subsection"><p id="p-5"><strong>CONCLUSIONS:</strong> In nonpineal locations, only a minority of histologically diagnosed primitive neuroectodermal tumors are embryonal tumors; therefore, high-grade glioma or ependymoma should be high on the radiographic differential. An understanding of molecularly defined tumor entities and their relative frequencies and locations will help the radiologist make more accurate predictions of the tumor types.</p></div></div><div class="section glossary" id="glossary-1"><h2 class="">ABBREVIATIONS:</h2><dl id="def-list-1"><dt id="def-item-1">ATRT<span class="def-item-dt-sep"></span></dt><dd>atypical teratoid/rhabdoid tumor<span class="def-item-dd-sep"></span></dd><dt id="def-item-2">EP<span class="def-item-dt-sep"></span></dt><dd>ependymoma<span class="def-item-dd-sep"></span></dd><dt id="def-item-3">ET<span class="def-item-dt-sep"></span></dt><dd>embryonal tumor<span class="def-item-dd-sep"></span></dd><dt id="def-item-4">ETMR<span class="def-item-dt-sep"></span></dt><dd>embryonal tumor with multilayered rosettes<span class="def-item-dd-sep"></span></dd><dt id="def-item-5">GBM<span class="def-item-dt-sep"></span></dt><dd>glioblastoma multiforme<span class="def-item-dd-sep"></span></dd><dt id="def-item-6">HGG<span class="def-item-dt-sep"></span></dt><dd>high-grade glioma<span class="def-item-dd-sep"></span></dd><dt id="def-item-7">HGNET<span class="def-item-dt-sep"></span></dt><dd>high-grade neuroepithelial tumor<span class="def-item-dd-sep"></span></dd><dt id="def-item-8">MB<span class="def-item-dt-sep"></span></dt><dd>medulloblastoma<span class="def-item-dd-sep"></span></dd><dt id="def-item-9">NOS<span class="def-item-dt-sep"></span></dt><dd>not otherwise specified<span class="def-item-dd-sep"></span></dd><dt id="def-item-10">PBL<span class="def-item-dt-sep"></span></dt><dd>pineoblastoma<span class="def-item-dd-sep"></span></dd><dt id="def-item-11">PNET<span class="def-item-dt-sep"></span></dt><dd>primitive neuroectodermal tumor</dd></dl></div><ul class="copyright-statement"><li class="fn" id="copyright-statement-1">© 2019 by American Journal of Neuroradiology</li></ul><div class="license" id="license-1"><p id="p-1">Indicates open access to non-subscribers at <a href="http://www.ajnr.org" rel="license">www.ajnr.org</a></p></div><span class="highwire-journal-article-marker-end"></span></div><span class="related-urls"></span></div><a href="http://ajnr-stage2.stage.highwire.org/content/40/11/1796.full" class="hw-link hw-link-article-full-text" data-icon-position="" data-hide-link-title="0">View Full Text</a></div>  </div>

  
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    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0"><span class="nlm-given-names">A.</span> <span class="nlm-surname">Jaju</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">E.I.</span> <span class="nlm-surname">Hwang</span></span>, <span class="highwire-citation-author" data-delta="2"><span class="nlm-given-names">M.</span> <span class="nlm-surname">Kool</span></span>, <span class="highwire-citation-author" data-delta="3"><span class="nlm-given-names">D.</span> <span class="nlm-surname">Capper</span></span>, <span class="highwire-citation-author" data-delta="4"><span class="nlm-given-names">L.</span> <span class="nlm-surname">Chavez</span></span>, <span class="highwire-citation-author" data-delta="5"><span class="nlm-given-names">S.</span> <span class="nlm-surname">Brabetz</span></span>, <span class="highwire-citation-author" data-delta="6"><span class="nlm-given-names">C.</span> <span class="nlm-surname">Billups</span></span>, <span class="highwire-citation-author" data-delta="7"><span class="nlm-given-names">Y.</span> <span class="nlm-surname">Li</span></span>, <span class="highwire-citation-author" data-delta="8"><span class="nlm-given-names">M.</span> <span class="nlm-surname">Fouladi</span></span>, <span class="highwire-citation-author" data-delta="9"><span class="nlm-given-names">R.J.</span> <span class="nlm-surname">Packer</span></span>, <span class="highwire-citation-author" data-delta="10"><span class="nlm-given-names">S.M.</span> <span class="nlm-surname">Pfister</span></span>, <span class="highwire-citation-author" data-delta="11"><span class="nlm-given-names">J.M.</span> <span class="nlm-surname">Olson</span></span>, <span class="highwire-citation-author" data-delta="12"><span class="nlm-given-names">L.A.</span> <span class="nlm-surname">Heier</span></span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-oa-ind highwire-cite-metadata"><i class="highwire-oa-indicator"></i> </span><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Nov 2019, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">40 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(11) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1796-1803; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A6253 </span></div>
  
  
  
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      <div class="highwire-cite-title">MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children's Oncology Group ACNS0332 Trial</div>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0"><span class="nlm-given-names">A.</span> <span class="nlm-surname">Jaju</span></span>, <span class="highwire-citation-author" data-delta="1"><span class="nlm-given-names">E.I.</span> <span class="nlm-surname">Hwang</span></span>, <span class="highwire-citation-author" data-delta="2"><span class="nlm-given-names">M.</span> <span class="nlm-surname">Kool</span></span>, <span class="highwire-citation-author" data-delta="3"><span class="nlm-given-names">D.</span> <span class="nlm-surname">Capper</span></span>, <span class="highwire-citation-author" data-delta="4"><span class="nlm-given-names">L.</span> <span class="nlm-surname">Chavez</span></span>, <span class="highwire-citation-author" data-delta="5"><span class="nlm-given-names">S.</span> <span class="nlm-surname">Brabetz</span></span>, <span class="highwire-citation-author" data-delta="6"><span class="nlm-given-names">C.</span> <span class="nlm-surname">Billups</span></span>, <span class="highwire-citation-author" data-delta="7"><span class="nlm-given-names">Y.</span> <span class="nlm-surname">Li</span></span>, <span class="highwire-citation-author" data-delta="8"><span class="nlm-given-names">M.</span> <span class="nlm-surname">Fouladi</span></span>, <span class="highwire-citation-author" data-delta="9"><span class="nlm-given-names">R.J.</span> <span class="nlm-surname">Packer</span></span>, <span class="highwire-citation-author" data-delta="10"><span class="nlm-given-names">S.M.</span> <span class="nlm-surname">Pfister</span></span>, <span class="highwire-citation-author" data-delta="11"><span class="nlm-given-names">J.M.</span> <span class="nlm-surname">Olson</span></span>, <span class="highwire-citation-author" data-delta="12"><span class="nlm-given-names">L.A.</span> <span class="nlm-surname">Heier</span></span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-oa-ind highwire-cite-metadata"><i class="highwire-oa-indicator"></i> </span><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Nov 2019, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">40 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(11) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1796-1803; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A6253 </span></div>
  
  
  
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