495 lines
No EOL
101 KiB
HTML
495 lines
No EOL
101 KiB
HTML
<?xml version="1.0" encoding="utf-8"?>
|
|
<!DOCTYPE html PUBLIC "-//W3C//DTD XHTML 1.0 Transitional//EN" "http://www.w3.org/TR/xhtml1/DTD/xhtml1-transitional.dtd">
|
|
<html xmlns="http://www.w3.org/1999/xhtml" xml:lang="en" lang="en">
|
|
|
|
<head><meta http-equiv="Content-Type" content="text/html; charset=utf-8" />
|
|
<!-- AppResources meta begin -->
|
|
<meta name="paf-app-resources" content="" />
|
|
<script type="text/javascript">var ncbi_startTime = new Date();</script>
|
|
|
|
<!-- AppResources meta end -->
|
|
|
|
<!-- TemplateResources meta begin -->
|
|
<meta name="paf_template" content="" />
|
|
|
|
<!-- TemplateResources meta end -->
|
|
|
|
<!-- Logger begin -->
|
|
<meta name="ncbi_db" content="books" /><meta name="ncbi_pdid" content="book-part" /><meta name="ncbi_acc" content="NBK552289" /><meta name="ncbi_domain" content="pdqcis" /><meta name="ncbi_report" content="record" /><meta name="ncbi_type" content="fulltext" /><meta name="ncbi_objectid" content="" /><meta name="ncbi_pcid" content="/NBK552289.13/" /><meta name="ncbi_pagename" content="Childhood Chordoma Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf" /><meta name="ncbi_bookparttype" content="chapter" /><meta name="ncbi_app" content="bookshelf" />
|
|
<!-- Logger end -->
|
|
|
|
<title>Childhood Chordoma Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf</title>
|
|
|
|
<!-- AppResources external_resources begin -->
|
|
<link rel="stylesheet" href="/core/jig/1.15.2/css/jig.min.css" /><script type="text/javascript" src="/core/jig/1.15.2/js/jig.min.js"></script>
|
|
|
|
<!-- AppResources external_resources end -->
|
|
|
|
<!-- Page meta begin -->
|
|
<meta name="robots" content="INDEX,FOLLOW,NOARCHIVE" /><meta name="citation_inbook_title" content="PDQ Cancer Information Summaries [Internet]" /><meta name="citation_title" content="Childhood Chordoma Treatment (PDQ®)" /><meta name="citation_publisher" content="National Cancer Institute (US)" /><meta name="citation_date" content="2019/12/23" /><meta name="citation_author" content="PDQ Pediatric Treatment Editorial Board" /><meta name="citation_pmid" content="31909945" /><meta name="citation_fulltext_html_url" content="https://www.ncbi.nlm.nih.gov/books/NBK552289/" /><meta name="citation_keywords" content="chordoma" /><meta name="citation_keywords" content="pediatric chordoma" /><link rel="schema.DC" href="http://purl.org/DC/elements/1.0/" /><meta name="DC.Title" content="Childhood Chordoma Treatment (PDQ®)" /><meta name="DC.Type" content="Text" /><meta name="DC.Publisher" content="National Cancer Institute (US)" /><meta name="DC.Contributor" content="PDQ Pediatric Treatment Editorial Board" /><meta name="DC.Date" content="2019/12/23" /><meta name="DC.Identifier" content="https://www.ncbi.nlm.nih.gov/books/NBK552289/" /><meta name="description" content="This evidence-based, expert-reviewed summary discusses the incidence, prognosis, clinical presentation, and treatment of pediatric chordoma." /><meta name="og:title" content="Childhood Chordoma Treatment (PDQ®)" /><meta name="og:type" content="book" /><meta name="og:description" content="This evidence-based, expert-reviewed summary discusses the incidence, prognosis, clinical presentation, and treatment of pediatric chordoma." /><meta name="og:url" content="https://www.ncbi.nlm.nih.gov/books/NBK552289/" /><meta name="og:site_name" content="NCBI Bookshelf" /><meta name="og:image" content="https://www.ncbi.nlm.nih.gov/corehtml/pmc/pmcgifs/bookshelf/thumbs/th-pdqcis-lrg.png" /><meta name="twitter:card" content="summary" /><meta name="twitter:site" content="@ncbibooks" /><meta name="bk-non-canon-loc" content="/books/n/pdqcis/CDR0000800112/" /><link rel="canonical" href="https://www.ncbi.nlm.nih.gov/books/NBK552289/" /><link rel="stylesheet" href="/corehtml/pmc/css/figpopup.css" type="text/css" media="screen" /><link rel="stylesheet" href="/corehtml/pmc/css/bookshelf/2.26/css/books.min.css" type="text/css" /><link rel="stylesheet" href="/corehtml/pmc/css/bookshelf/2.26/css/books_print.min.css" type="text/css" media="print" /><style type="text/css">p a.figpopup{display:inline !important} .bk_tt {font-family: monospace} .first-line-outdent .bk_ref {display: inline} .body-content h2, .body-content .h2 {border-bottom: 1px solid #97B0C8} .body-content h2.inline {border-bottom: none} a.page-toc-label , .jig-ncbismoothscroll a {text-decoration:none;border:0 !important} .temp-labeled-list .graphic {display:inline-block !important} .temp-labeled-list img{width:100%}</style><script type="text/javascript" src="/corehtml/pmc/js/jquery.hoverIntent.min.js"> </script><script type="text/javascript" src="/corehtml/pmc/js/common.min.js?_=3.18"> </script><script type="text/javascript" src="/corehtml/pmc/js/large-obj-scrollbars.min.js"> </script><script type="text/javascript">window.name="mainwindow";</script><script type="text/javascript" src="/corehtml/pmc/js/bookshelf/2.26/book-toc.min.js"> </script><script type="text/javascript" src="/corehtml/pmc/js/bookshelf/2.26/books.min.js"> </script><meta name="book-collection" content="NONE" />
|
|
|
|
<!-- Page meta end -->
|
|
<link rel="shortcut icon" href="//www.ncbi.nlm.nih.gov/favicon.ico" /><meta name="ncbi_phid" content="CE8B59A27C9809B10000000000470043.m_13" />
|
|
<meta name='referrer' content='origin-when-cross-origin'/><link type="text/css" rel="stylesheet" href="//static.pubmed.gov/portal/portal3rc.fcgi/4216699/css/3852956/3985586/3808861/4121862/3974050/3917732/251717/4216701/14534/45193/4113719/3849091/3984811/3751656/4033350/3840896/3577051/3852958/4008682/4207974/4206132/4062871/12930/3964959/3854974/36029/4128070/9685/3549676/3609192/3609193/3609213/3395586.css" /><link type="text/css" rel="stylesheet" href="//static.pubmed.gov/portal/portal3rc.fcgi/4216699/css/3411343/3882866.css" media="print" /></head>
|
|
<body class="book-part">
|
|
<div class="grid">
|
|
<div class="col twelve_col nomargin shadow">
|
|
<!-- System messages like service outage or JS required; this is handled by the TemplateResources portlet -->
|
|
<div class="sysmessages">
|
|
<noscript>
|
|
<p class="nojs">
|
|
<strong>Warning:</strong>
|
|
The NCBI web site requires JavaScript to function.
|
|
<a href="/guide/browsers/#enablejs" title="Learn how to enable JavaScript" target="_blank">more...</a>
|
|
</p>
|
|
</noscript>
|
|
</div>
|
|
<!--/.sysmessage-->
|
|
<div class="wrap">
|
|
<div class="page">
|
|
<div class="top">
|
|
<div id="universal_header">
|
|
<section class="usa-banner">
|
|
<div class="usa-accordion">
|
|
<header class="usa-banner-header">
|
|
<div class="usa-grid usa-banner-inner">
|
|
<img src="https://www.ncbi.nlm.nih.gov/coreutils/uswds/img/favicons/favicon-57.png" alt="U.S. flag" />
|
|
<p>An official website of the United States government</p>
|
|
<button class="non-usa-accordion-button usa-banner-button" aria-expanded="false" aria-controls="gov-banner-top" type="button">
|
|
<span class="usa-banner-button-text">Here's how you know</span>
|
|
</button>
|
|
</div>
|
|
</header>
|
|
<div class="usa-banner-content usa-grid usa-accordion-content" id="gov-banner-top" aria-hidden="true">
|
|
<div class="usa-banner-guidance-gov usa-width-one-half">
|
|
<img class="usa-banner-icon usa-media_block-img" src="https://www.ncbi.nlm.nih.gov/coreutils/uswds/img/icon-dot-gov.svg" alt="Dot gov" />
|
|
<div class="usa-media_block-body">
|
|
<p>
|
|
<strong>The .gov means it's official.</strong>
|
|
<br />
|
|
Federal government websites often end in .gov or .mil. Before
|
|
sharing sensitive information, make sure you're on a federal
|
|
government site.
|
|
</p>
|
|
</div>
|
|
</div>
|
|
<div class="usa-banner-guidance-ssl usa-width-one-half">
|
|
<img class="usa-banner-icon usa-media_block-img" src="https://www.ncbi.nlm.nih.gov/coreutils/uswds/img/icon-https.svg" alt="Https" />
|
|
<div class="usa-media_block-body">
|
|
<p>
|
|
<strong>The site is secure.</strong>
|
|
<br />
|
|
The <strong>https://</strong> ensures that you are connecting to the
|
|
official website and that any information you provide is encrypted
|
|
and transmitted securely.
|
|
</p>
|
|
</div>
|
|
</div>
|
|
</div>
|
|
</div>
|
|
</section>
|
|
<div class="usa-overlay"></div>
|
|
<header class="ncbi-header" role="banner" data-section="Header">
|
|
|
|
<div class="usa-grid">
|
|
<div class="usa-width-one-whole">
|
|
|
|
<div class="ncbi-header__logo">
|
|
<a href="/" class="logo" aria-label="NCBI Logo" data-ga-action="click_image" data-ga-label="NIH NLM Logo">
|
|
<img src="https://www.ncbi.nlm.nih.gov/coreutils/nwds/img/logos/AgencyLogo.svg" alt="NIH NLM Logo" />
|
|
</a>
|
|
</div>
|
|
|
|
<div class="ncbi-header__account">
|
|
<a id="account_login" href="https://account.ncbi.nlm.nih.gov" class="usa-button header-button" style="display:none" data-ga-action="open_menu" data-ga-label="account_menu">Log in</a>
|
|
<button id="account_info" class="header-button" style="display:none" aria-controls="account_popup" type="button">
|
|
<span class="fa fa-user" aria-hidden="true">
|
|
<svg xmlns="http://www.w3.org/2000/svg" viewBox="0 0 24 24" width="20px" height="20px">
|
|
<g style="fill: #fff">
|
|
<ellipse cx="12" cy="8" rx="5" ry="6"></ellipse>
|
|
<path d="M21.8,19.1c-0.9-1.8-2.6-3.3-4.8-4.2c-0.6-0.2-1.3-0.2-1.8,0.1c-1,0.6-2,0.9-3.2,0.9s-2.2-0.3-3.2-0.9 C8.3,14.8,7.6,14.7,7,15c-2.2,0.9-3.9,2.4-4.8,4.2C1.5,20.5,2.6,22,4.1,22h15.8C21.4,22,22.5,20.5,21.8,19.1z"></path>
|
|
</g>
|
|
</svg>
|
|
</span>
|
|
<span class="username desktop-only" aria-hidden="true" id="uname_short"></span>
|
|
<span class="sr-only">Show account info</span>
|
|
</button>
|
|
</div>
|
|
|
|
<div class="ncbi-popup-anchor">
|
|
<div class="ncbi-popup account-popup" id="account_popup" aria-hidden="true">
|
|
<div class="ncbi-popup-head">
|
|
<button class="ncbi-close-button" data-ga-action="close_menu" data-ga-label="account_menu" type="button">
|
|
<span class="fa fa-times">
|
|
<svg xmlns="http://www.w3.org/2000/svg" viewBox="0 0 48 48" width="24px" height="24px">
|
|
<path d="M38 12.83l-2.83-2.83-11.17 11.17-11.17-11.17-2.83 2.83 11.17 11.17-11.17 11.17 2.83 2.83 11.17-11.17 11.17 11.17 2.83-2.83-11.17-11.17z"></path>
|
|
</svg>
|
|
</span>
|
|
<span class="usa-sr-only">Close</span></button>
|
|
<h4>Account</h4>
|
|
</div>
|
|
<div class="account-user-info">
|
|
Logged in as:<br />
|
|
<b><span class="username" id="uname_long">username</span></b>
|
|
</div>
|
|
<div class="account-links">
|
|
<ul class="usa-unstyled-list">
|
|
<li><a id="account_myncbi" href="/myncbi/" class="set-base-url" data-ga-action="click_menu_item" data-ga-label="account_myncbi">Dashboard</a></li>
|
|
<li><a id="account_pubs" href="/myncbi/collections/bibliography/" class="set-base-url" data-ga-action="click_menu_item" data-ga-label="account_pubs">Publications</a></li>
|
|
<li><a id="account_settings" href="/account/settings/" class="set-base-url" data-ga-action="click_menu_item" data-ga-label="account_settings">Account settings</a></li>
|
|
<li><a id="account_logout" href="/account/signout/" class="set-base-url" data-ga-action="click_menu_item" data-ga-label="account_logout">Log out</a></li>
|
|
</ul>
|
|
</div>
|
|
</div>
|
|
</div>
|
|
|
|
</div>
|
|
</div>
|
|
</header>
|
|
<div role="navigation" aria-label="access keys">
|
|
<a id="nws_header_accesskey_0" href="https://www.ncbi.nlm.nih.gov/guide/browsers/#ncbi_accesskeys" class="usa-sr-only" accesskey="0" tabindex="-1">Access keys</a>
|
|
<a id="nws_header_accesskey_1" href="https://www.ncbi.nlm.nih.gov" class="usa-sr-only" accesskey="1" tabindex="-1">NCBI Homepage</a>
|
|
<a id="nws_header_accesskey_2" href="/myncbi/" class="set-base-url usa-sr-only" accesskey="2" tabindex="-1">MyNCBI Homepage</a>
|
|
<a id="nws_header_accesskey_3" href="#maincontent" class="usa-sr-only" accesskey="3" tabindex="-1">Main Content</a>
|
|
<a id="nws_header_accesskey_4" href="#" class="usa-sr-only" accesskey="4" tabindex="-1">Main Navigation</a>
|
|
</div>
|
|
<section data-section="Alerts">
|
|
<div class="ncbi-alerts-placeholder"></div>
|
|
</section>
|
|
</div>
|
|
<div class="header">
|
|
<div class="res_logo"><h1 class="res_name"><a href="/books/" title="Bookshelf home">Bookshelf</a></h1><h2 class="res_tagline"></h2></div>
|
|
<div class="search"><form method="get" action="/books/"><div class="search_form"><label for="database" class="offscreen_noflow">Search database</label><select id="database"><optgroup label="Recent"><option value="books" selected="selected" data-ac_dict="bookshelf-search">Books</option><option value="nlmcatalog">NLM Catalog</option><option value="pcsubstance">PubChem Substance</option><option value="pubmed" class="last">PubMed</option></optgroup><optgroup label="All"><option value="gquery">All Databases</option><option value="assembly">Assembly</option><option value="biocollections">Biocollections</option><option value="bioproject">BioProject</option><option value="biosample">BioSample</option><option value="books" data-ac_dict="bookshelf-search">Books</option><option value="clinvar">ClinVar</option><option value="cdd">Conserved Domains</option><option value="gap">dbGaP</option><option value="dbvar">dbVar</option><option value="gene">Gene</option><option value="genome">Genome</option><option value="gds">GEO DataSets</option><option value="geoprofiles">GEO Profiles</option><option value="gtr">GTR</option><option value="ipg">Identical Protein Groups</option><option value="medgen">MedGen</option><option value="mesh">MeSH</option><option value="nlmcatalog">NLM Catalog</option><option value="nuccore">Nucleotide</option><option value="omim">OMIM</option><option value="pmc">PMC</option><option value="protein">Protein</option><option value="proteinclusters">Protein Clusters</option><option value="protfam">Protein Family Models</option><option value="pcassay">PubChem BioAssay</option><option value="pccompound">PubChem Compound</option><option value="pcsubstance">PubChem Substance</option><option value="pubmed">PubMed</option><option value="snp">SNP</option><option value="sra">SRA</option><option value="structure">Structure</option><option value="taxonomy">Taxonomy</option><option value="toolkit">ToolKit</option><option value="toolkitall">ToolKitAll</option><option value="toolkitbookgh">ToolKitBookgh</option></optgroup></select><div class="nowrap"><label for="term" class="offscreen_noflow" accesskey="/">Search term</label><div class="nowrap"><input type="text" name="term" id="term" title="Search Books. Use up and down arrows to choose an item from the autocomplete." value="" class="jig-ncbiclearbutton jig-ncbiautocomplete" data-jigconfig="dictionary:'bookshelf-search',disableUrl:'NcbiSearchBarAutoComplCtrl'" autocomplete="off" data-sbconfig="ds:'no',pjs:'no',afs:'no'" /></div><button id="search" type="submit" class="button_search nowrap" cmd="go">Search</button></div></div></form><ul class="searchlinks inline_list"><li>
|
|
<a href="/books/browse/">Browse Titles</a>
|
|
</li><li>
|
|
<a href="/books/advanced/">Advanced</a>
|
|
</li><li class="help">
|
|
<a href="/books/NBK3833/">Help</a>
|
|
</li><li class="disclaimer">
|
|
<a target="_blank" data-ga-category="literature_resources" data-ga-action="link_click" data-ga-label="disclaimer_link" href="https://www.ncbi.nlm.nih.gov/books/about/disclaimer/">Disclaimer</a>
|
|
</li></ul></div>
|
|
</div>
|
|
|
|
|
|
|
|
<!--<component id="Page" label="headcontent"/>-->
|
|
|
|
</div>
|
|
<div class="content">
|
|
<!-- site messages -->
|
|
<!-- Custom content 1 -->
|
|
<div class="col1">
|
|
|
|
</div>
|
|
|
|
<div class="container">
|
|
<div id="maincontent" class="content eight_col col">
|
|
<!-- Custom content in the left column above book nav -->
|
|
<div class="col2">
|
|
|
|
</div>
|
|
|
|
<!-- Book content -->
|
|
|
|
|
|
<!-- Custom content between navigation and content -->
|
|
<div class="col3">
|
|
|
|
</div>
|
|
|
|
<div class="document">
|
|
<div class="pre-content"><div><div class="bk_prnt"><p class="small">NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.</p><p>PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. </p></div><div class="iconblock clearfix whole_rhythm no_top_margin bk_noprnt"><a class="img_link icnblk_img" title="Table of Contents Page" href="/books/n/pdqcis/"><img class="source-thumb" src="/corehtml/pmc/pmcgifs/bookshelf/thumbs/th-pdqcis-lrg.png" alt="Cover of PDQ Cancer Information Summaries" height="100px" width="80px" /></a><div class="icnblk_cntnt eight_col"><h2>PDQ Cancer Information Summaries [Internet].</h2><a data-jig="ncbitoggler" href="#__NBK552289_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK552289_dtls__"><div>Bethesda (MD): <a href="http://www.cancer.gov/" ref="pagearea=page-banner&targetsite=external&targetcat=link&targettype=publisher">National Cancer Institute (US)</a>; 2002-.</div></div><div class="half_rhythm"></div><div class="bk_noprnt"><form method="get" action="/books/n/pdqcis/" id="bk_srch"><div class="bk_search"><label for="bk_term" class="offscreen_noflow">Search term</label><input type="text" title="Search this book" id="bk_term" name="term" value="" data-jig="ncbiclearbutton" /> <input type="submit" class="jig-ncbibutton" value="Search this book" submit="false" style="padding: 0.1em 0.4em;" /></div></form></div></div></div></div></div>
|
|
<div class="main-content lit-style" itemscope="itemscope" itemtype="http://schema.org/CreativeWork"><div class="meta-content fm-sec"><h1 id="_NBK552289_"><span class="title" itemprop="name">Childhood Chordoma Treatment (PDQ®)</span></h1><div class="subtitle whole_rhythm">Health Professional Version</div><p class="contrib-group"><span itemprop="author">PDQ Pediatric Treatment Editorial Board</span>.</p><p class="small">Published online: September 16, 2024.</p><p class="small">Created: <span itemprop="datePublished">December 23, 2019</span>.</p></div><div class="jig-ncbiinpagenav body-content whole_rhythm" data-jigconfig="allHeadingLevels: ['h2'],smoothScroll: false" itemprop="text"><div id="_abs_rndgid_" itemprop="description"><p id="CDR0000800112__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric chordoma. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.</p><p id="CDR0000800112__1557">This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).</p></div><div id="CDR0000800112__996"><h2 id="_CDR0000800112__996_">Incidence</h2><p id="CDR0000800112__997">Chordoma is a very rare tumor of bone. It arises from remnants of the notochord within the clivus, spinal vertebrae, or sacrum. The most common site in children is the cranium.[<a class="bk_pop" href="#CDR0000800112_rl_996_1">1</a>] The incidence in the United States is approximately 1 case per 1 million people per year. Only 5% of all chordomas occur in patients younger than 20 years.[<a class="bk_pop" href="#CDR0000800112_rl_996_2">2</a>,<a class="bk_pop" href="#CDR0000800112_rl_996_3">3</a>] Most pediatric patients have the classical or chondroid variant of chordoma, while the dedifferentiated variant is rare in children.[<a class="bk_pop" href="#CDR0000800112_rl_996_2">2</a>,<a class="bk_pop" href="#CDR0000800112_rl_996_4">4</a>] </p><div id="CDR0000800112_rl_996"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800112_rl_996_1">Sebro R, DeLaney T, Hornicek F, et al.: Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas. BMC Med Imaging 16 (1): 53, 2016. [<a href="/pmc/articles/PMC5016865/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5016865</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/27609115" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 27609115</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_996_2">Hoch BL, Nielsen GP, Liebsch NJ, et al.: Base of skull chordomas in children and adolescents: a clinicopathologic study of 73 cases. Am J Surg Pathol 30 (7): 811-8, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16819322" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16819322</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_996_3">Lau CS, Mahendraraj K, Ward A, et al.: Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011). Pediatr Neurosurg 51 (3): 127-36, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26881831" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26881831</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_996_4">McMaster ML, Goldstein AM, Bromley CM, et al.: Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control 12 (1): 1-11, 2001. [<a href="https://pubmed.ncbi.nlm.nih.gov/11227920" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11227920</span></a>]</div></li></ol></div></div><div id="CDR0000800112__1000"><h2 id="_CDR0000800112__1000_">Clinical Presentation and Diagnosis</h2><p id="CDR0000800112__1001">Patients with chordomas usually present with pain (headache or sacrum) or diplopia. Patients may also present with or without neurological deficits such as cranial or other nerve impairment.[<a class="bk_pop" href="#CDR0000800112_rl_1000_1">1</a>] </p><p id="CDR0000800112__2476">The diagnosis of a chordoma is straightforward when the typical physaliferous (soap bubble–bearing) cells are present. The differential diagnosis is sometimes difficult and includes dedifferentiated chordoma and chondrosarcoma. Childhood chordoma has been associated with tuberous sclerosis complex.[<a class="bk_pop" href="#CDR0000800112_rl_1000_2">2</a>]</p><div id="CDR0000800112_rl_1000"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800112_rl_1000_1">John L, Smith H, Ilanchezhian M, et al.: The NIH pediatric/young adult chordoma clinic and natural history study: Making advances in a very rare tumor. Pediatr Blood Cancer : e30358, 2023. [<a href="/pmc/articles/PMC10739575/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC10739575</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/37347686" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 37347686</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1000_2">McMaster ML, Goldstein AM, Parry DM: Clinical features distinguish childhood chordoma associated with tuberous sclerosis complex (TSC) from chordoma in the general paediatric population. J Med Genet 48 (7): 444-9, 2011. [<a href="/pmc/articles/PMC3235000/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3235000</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/21266383" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21266383</span></a>]</div></li></ol></div></div><div id="CDR0000800112__998"><h2 id="_CDR0000800112__998_">Prognosis and Molecular Features</h2><p id="CDR0000800112__999">Younger children with chordomas appear to have a worse outlook than older patients.[<a class="bk_pop" href="#CDR0000800112_rl_998_1">1</a>-<a class="bk_pop" href="#CDR0000800112_rl_998_6">6</a>] The survival rate ranges from about 50% to 80% for children and adolescents with cranial chordomas.[<a class="bk_pop" href="#CDR0000800112_rl_998_2">2</a>,<a class="bk_pop" href="#CDR0000800112_rl_998_3">3</a>,<a class="bk_pop" href="#CDR0000800112_rl_998_5">5</a>] However, in a National Cancer Database review, the overall survival (OS) of pediatric and adult patients with cranial chordomas was similar (70% at 10 years).[<a class="bk_pop" href="#CDR0000800112_rl_998_7">7</a>]</p><ul id="CDR0000800112__2461"><li class="half_rhythm"><div class="half_rhythm">A retrospective literature review and review of institutional patients identified 682 patients with spinal chordomas. The median age of patients was 57 years.[<a class="bk_pop" href="#CDR0000800112_rl_998_8">8</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>]<ul id="CDR0000800112__2462"><li class="half_rhythm"><div>Age younger than 18 years, sacral spine tumor location, dedifferentiated pathology, and treatment with chemotherapy were associated with a lower probability for progression-free survival (PFS). </div></li><li class="half_rhythm"><div>Younger age (<18 years), older age (>65 years), bladder or bowel dysfunction at presentation, dedifferentiated pathology, disease recurrence or progression, and metastatic disease were associated with a worse OS. </div></li></ul></div></li><li class="half_rhythm"><div class="half_rhythm"> Histopathology is also an important prognostic factor. Patients who have tumors with typical or chondroid pathology have worse outcomes than patients who have tumors with classical pathology.[<a class="bk_pop" href="#CDR0000800112_rl_998_9">9</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">A multicenter retrospective study identified 40 children with chordomas (median age, 12 years).[<a class="bk_pop" href="#CDR0000800112_rl_998_10">10</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>]<ul id="CDR0000800112__2463"><li class="half_rhythm"><div> Most of the patients had the histologically classical form of chordoma (45.5%).</div></li><li class="half_rhythm"><div>Most of the chordomas were located at the skull base (72.5%).</div></li><li class="half_rhythm"><div> The OS rates were 66.6% at 5 years and 58.6% at 10 years.</div></li><li class="half_rhythm"><div> The PFS rates were 55.7% at 5 years and 52% at 10 years. </div></li><li class="half_rhythm"><div>Total resection correlated with a better outcome (log-rank <i>P</i> = .04 for OS and PFS).</div></li><li class="half_rhythm"><div> Loss of BAF47 immunoexpression appeared to be a significant independent adverse prognostic factor (<i>P</i> = .033 for PFS).</div></li></ul></div></li><li class="half_rhythm"><div class="half_rhythm">A retrospective analysis identified seven children with poorly differentiated chordomas.[<a class="bk_pop" href="#CDR0000800112_rl_998_11">11</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>]<ul id="CDR0000800112__2464"><li class="half_rhythm"><div> The median survival of these patients was 9 months.</div></li><li class="half_rhythm"><div> All poorly differentiated chordomas showed loss of <i>SMARCB1</i> expression by immunohistochemistry. Copy number profiles were derived from intensity measures of the methylation probes and indicated 22q losses affecting the <i>SMARCB1</i> region in all poorly differentiated chordomas.</div></li></ul></div><div class="half_rhythm">Inactivation of the <i>SMARCB1</i> gene is common in poorly differentiated chordomas of childhood, and it is associated with a poor prognosis.[<a class="bk_pop" href="#CDR0000800112_rl_998_11">11</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">The National Cancer Institute (NCI) analyzed germline DNA from a cohort of 24 patients with chordomas who were referred to the NCI (age range, 5–57 years).[<a class="bk_pop" href="#CDR0000800112_rl_998_12">12</a>]<ul id="CDR0000800112__2504"><li class="half_rhythm"><div> Pathogenic variants in cancer predisposition genes were identified in 9 of the 24 patients (38%).</div></li></ul></div></li></ul><div id="CDR0000800112_rl_998"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800112_rl_998_1">Coffin CM, Swanson PE, Wick MR, et al.: Chordoma in childhood and adolescence. A clinicopathologic analysis of 12 cases. Arch Pathol Lab Med 117 (9): 927-33, 1993. [<a href="https://pubmed.ncbi.nlm.nih.gov/8368907" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 8368907</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_2">Borba LA, Al-Mefty O, Mrak RE, et al.: Cranial chordomas in children and adolescents. J Neurosurg 84 (4): 584-91, 1996. [<a href="https://pubmed.ncbi.nlm.nih.gov/8613849" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 8613849</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_3">Hoch BL, Nielsen GP, Liebsch NJ, et al.: Base of skull chordomas in children and adolescents: a clinicopathologic study of 73 cases. Am J Surg Pathol 30 (7): 811-8, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16819322" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16819322</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_4">Jian BJ, Bloch OG, Yang I, et al.: A comprehensive analysis of intracranial chordoma and survival: a systematic review. Br J Neurosurg 25 (4): 446-53, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21749184" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21749184</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_5">Yasuda M, Bresson D, Chibbaro S, et al.: Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients. Neurosurg Rev 35 (2): 171-82; discussion 182-3, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/21863225" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21863225</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_6">Chambers KJ, Lin DT, Meier J, et al.: Incidence and survival patterns of cranial chordoma in the United States. Laryngoscope 124 (5): 1097-102, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/24122844" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24122844</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_7">Xu JC, Lehrich BM, Yasaka TM, et al.: Characteristics and overall survival in pediatric versus adult skull base chordoma: a population-based study. Childs Nerv Syst 37 (6): 1901-1908, 2021. [<a href="https://pubmed.ncbi.nlm.nih.gov/33459820" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33459820</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_8">Zhou J, Sun J, Bai HX, et al.: Prognostic Factors in Patients With Spinal Chordoma: An Integrative Analysis of 682 Patients. Neurosurgery 81 (5): 812-823, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28368502" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28368502</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_9">Tsitouras V, Wang S, Dirks P, et al.: Management and outcome of chordomas in the pediatric population: The Hospital for Sick Children experience and review of the literature. J Clin Neurosci 34: 169-176, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/27590862" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 27590862</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_10">Beccaria K, Tauziède-Espariat A, Monnien F, et al.: Pediatric Chordomas: Results of a Multicentric Study of 40 Children and Proposal for a Histopathological Prognostic Grading System and New Therapeutic Strategies. J Neuropathol Exp Neurol 77 (3): 207-215, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/29361006" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29361006</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_11">Hasselblatt M, Thomas C, Hovestadt V, et al.: Poorly differentiated chordoma with SMARCB1/INI1 loss: a distinct molecular entity with dismal prognosis. Acta Neuropathol 132 (1): 149-51, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/27067307" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 27067307</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_998_12">Raygada M, John L, Liu A, et al.: Germline findings in cancer predisposing genes from a small cohort of chordoma patients. J Cancer Res Clin Oncol 150 (5): 227, 2024. [<a href="/pmc/articles/PMC11068663/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC11068663</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/38700789" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 38700789</span></a>]</div></li></ol></div></div><div id="CDR0000800112__1864"><h2 id="_CDR0000800112__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000800112__1864_md_3">Cancer in children and adolescents is rare, although the overall incidence has slowly increased since 1975.[<a class="bk_pop" href="#CDR0000800112_rl_1864_1">1</a>] Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team approach incorporates the skills
|
|
of the following pediatric specialists and others to ensure that children receive treatment, supportive care, and rehabilitation
|
|
to achieve optimal survival and quality of life:</p><ul id="CDR0000800112__1864_md_4"><li class="half_rhythm"><div>Primary care physicians.</div></li><li class="half_rhythm"><div>Pediatric surgeons.</div></li><li class="half_rhythm"><div>Pathologists.</div></li><li class="half_rhythm"><div>Pediatric radiation
|
|
oncologists.</div></li><li class="half_rhythm"><div>Pediatric medical oncologists and hematologists.</div></li><li class="half_rhythm"><div>Ophthalmologists.</div></li><li class="half_rhythm"><div> Rehabilitation
|
|
specialists.</div></li><li class="half_rhythm"><div>Pediatric oncology nurses.</div></li><li class="half_rhythm"><div>Social workers.</div></li><li class="half_rhythm"><div>Child-life professionals.</div></li><li class="half_rhythm"><div>Psychologists.</div></li><li class="half_rhythm"><div>Nutritionists.</div></li></ul><p id="CDR0000800112__1864_md_5">For specific information about supportive care for children and adolescents with cancer, see the summaries on <a href="https://www.cancer.gov/publications/pdq/information-summaries/supportive-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Supportive and Palliative Care</a>.</p><p id="CDR0000800112__1864_md_6">The American Academy of Pediatrics has outlined guidelines for
|
|
pediatric cancer centers and their role in the treatment of children and adolescents
|
|
with cancer.[<a class="bk_pop" href="#CDR0000800112_rl_1864_2">2</a>] At
|
|
these centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity
|
|
to participate is offered to most patients and their families. Clinical
|
|
trials for children and adolescents diagnosed with cancer are generally
|
|
designed to compare potentially better therapy with current standard therapy. Other types of clinical trials test novel therapies when there is no standard therapy for a cancer diagnosis. Most of the progress in identifying curative
|
|
therapies for childhood cancers has been achieved through clinical trials.
|
|
Information about ongoing clinical trials is available from the <a href="https://www.cancer.gov/research/participate/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>.</p><p id="CDR0000800112__1864_md_7">Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[<a class="bk_pop" href="#CDR0000800112_rl_1864_3">3</a>-<a class="bk_pop" href="#CDR0000800112_rl_1864_5">5</a>] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. For information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors, see <a href="/books/n/pdqcis/CDR0000343584/">Late Effects of Treatment for Childhood Cancer</a>.</p><p id="CDR0000800112__1864_md_8">Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years.[<a class="bk_pop" href="#CDR0000800112_rl_1864_6">6</a>] The U.S. <a href="https://www.congress.gov/107/plaws/publ280/PLAW-107publ280.pdf" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Rare Diseases Act of 2002</a> defines a rare disease as one that affects populations smaller than 200,000 people in the United States. Therefore, all pediatric cancers are considered rare.</p><p id="CDR0000800112__1864_md_9">The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[<a class="bk_pop" href="#CDR0000800112_rl_1864_7">7</a>,<a class="bk_pop" href="#CDR0000800112_rl_1864_8">8</a>] In children and adolescents, the designation of a rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000800112__1864_md_10"><li class="half_rhythm"><div class="half_rhythm">A consensus effort between the European Union Joint Action on Rare Cancers and the European Cooperative Study Group for Rare Pediatric Cancers estimated that 11% of all cancers in patients younger than 20 years could be categorized as very rare. This consensus group defined very rare cancers as those with annual incidences of fewer than two cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than two cases per 1 million people were also included in the very rare group due to a lack of knowledge and expertise in the management of these tumors.[<a class="bk_pop" href="#CDR0000800112_rl_1864_9">9</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">The Children's Oncology Group defines rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancers, melanomas and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinomas, nasopharyngeal carcinomas, and most adult-type carcinomas such as breast cancers and colorectal cancers).[<a class="bk_pop" href="#CDR0000800112_rl_1864_10">10</a>] These diagnoses account for about 5% of the cancers diagnosed in children aged 0 to 14 years and about 27% of the cancers diagnosed in adolescents aged 15 to 19 years.[<a class="bk_pop" href="#CDR0000800112_rl_1864_4">4</a>]</div><div class="half_rhythm"> Most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers diagnosed in children aged 0 to 14 years and 9.3% of the cancers diagnosed in adolescents aged 15 to 19 years.</div></li></ul><p id="CDR0000800112__1864_md_12">These rare cancers are extremely challenging to study because of the relatively few patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the small number of clinical trials for adolescents with rare cancers.</p><div id="CDR0000800112_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800112_rl_1864_1">Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010. [<a href="/pmc/articles/PMC2881732/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2881732</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20404250" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20404250</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1864_2">American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. <a href="https://pediatrics.aappublications.org/content/134/2/410" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Also available online</a>. Last accessed August 23, 2024.</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1864_3">Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014. [<a href="/pmc/articles/PMC4136455/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4136455</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/24853691" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24853691</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1864_4">National Cancer Institute: NCCR*Explorer: An interactive website for NCCR cancer statistics. Bethesda, MD: National Cancer Institute. <a href="https://NCCRExplorer.ccdi.cancer.gov/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online</a>. Last accessed August 23, 2024.</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1864_5">Surveillance Research Program, National Cancer Institute: SEER*Explorer: An interactive website for SEER cancer statistics. Bethesda, MD: National Cancer Institute. <a href="https://seer.cancer.gov/statistics-network/explorer/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online</a>. Last accessed September 5, 2024.</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1864_6">Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr. [<a href="https://pubmed.ncbi.nlm.nih.gov/24488779" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24488779</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1864_7">Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28687376" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28687376</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1864_8">DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28542893" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28542893</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1864_9">Ferrari A, Brecht IB, Gatta G, et al.: Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 110: 120-126, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/30785015" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30785015</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1864_10">Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010. [<a href="/pmc/articles/PMC3020699/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3020699</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20956621" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20956621</span></a>]</div></li></ol></div></div><div id="CDR0000800112__1002"><h2 id="_CDR0000800112__1002_">Treatment of Childhood Chordoma</h2><p id="CDR0000800112__2292">One report described the value of using a multidisciplinary clinic for patients with these very rare tumors.[<a class="bk_pop" href="#CDR0000800112_rl_1002_1">1</a>] Treatment options for childhood chordoma include the following:</p><ol id="CDR0000800112__2293"><li class="half_rhythm"><div><a href="#CDR0000800112__2466">Surgery with or without radiation therapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000800112__2470">Tyrosine kinase inhibitor (TKI) therapy</a>.</div></li></ol><div id="CDR0000800112__2466"><h3>Surgery With or Without Radiation Therapy</h3><p id="CDR0000800112__1003">Standard treatment includes surgery and external radiation therapy, often proton-beam radiation.[<a class="bk_pop" href="#CDR0000800112_rl_1002_2">2</a>,<a class="bk_pop" href="#CDR0000800112_rl_1002_3">3</a>] Surgery is often not curative in children and adolescents because of the likelihood of the chordomas arising in the skull base, rather than in the sacrum, making them relatively inaccessible for complete surgical excision. However, if gross-total resection can be achieved, outcome is improved.[<a class="bk_pop" href="#CDR0000800112_rl_1002_4">4</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>]</p><p id="CDR0000800112__2294"> The best results have been obtained using proton-beam therapy (<a href="/books/n/pdqcis/glossary/def-item/glossary_CDR0000534234/" class="def">charged-particle radiation therapy</a>) because these tumors are relatively radiation resistant, and radiation-dose conformality with protons allows for higher tumor doses while sparing adjacent critical normal tissues.[<a class="bk_pop" href="#CDR0000800112_rl_1002_5">5</a>-<a class="bk_pop" href="#CDR0000800112_rl_1002_8">8</a>]; [<a class="bk_pop" href="#CDR0000800112_rl_1002_2">2</a>,<a class="bk_pop" href="#CDR0000800112_rl_1002_9">9</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>]; [<a class="bk_pop" href="#CDR0000800112_rl_1002_10">10</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810037/" class="def">Level of evidence C2</a>] </p><p id="CDR0000800112__2467">Evidence (surgery and/or radiation therapy):</p><ol id="CDR0000800112__2468"><li class="half_rhythm"><div>In a retrospective study of 20 children with skull-based chordomas, the median age at diagnosis was 12 years. The most common presenting symptoms were diplopia, headache, and swallowing difficulties.[<a class="bk_pop" href="#CDR0000800112_rl_1002_11">11</a>] Five patients had locally recurrent tumors. Twelve patients underwent surgery with an endoscopic endonasal approach alone, and eight patients underwent other procedures. All but two patients received radiation therapy. Fourteen patients had gross-total resections, ten of whom developed surgical complications.<ul id="CDR0000800112__2469"><li class="half_rhythm"><div> No differences in recurrence rates were seen between patients who presented with a new diagnosis and patients who had recurrent disease or between patients who underwent a gross-total resection and patients who underwent a near-total resection.</div></li><li class="half_rhythm"><div> Of patients who received postoperative radiation therapy, none had a recurrence.</div></li><li class="half_rhythm"><div> Comparatively, of the eleven patients who either did not receive radiation therapy or were treated preoperatively, four had a recurrence (<i>P</i> = .09).</div></li><li class="half_rhythm"><div> Three patients developed distant metastases, and three patients died of disease.</div></li><li class="half_rhythm"><div> A high Ki-67 index was more prevalent among patients with dedifferentiated chordomas. Two of the three patients who died had an elevated Ki-67 index.</div></li></ul></div></li><li class="half_rhythm"><div>Pediatric patients with base of skull chordomas were treated with proton-beam therapy or a combined proton/photon approach (proton-based; most received 80% proton/20% photon) at the Massachusetts General Hospital from 1981 to 2021. Of 204 patients, the median age at diagnosis was 11.1 years (range, 1–21 years).[<a class="bk_pop" href="#CDR0000800112_rl_1002_12">12</a>] <ul id="CDR0000800112__2481"><li class="half_rhythm"><div>The chordomas presented in the upper and/or middle clivus in 59% of the patients, lower clivus in 36%, craniocervical junction in 4%, and nasal cavity in 1%. </div></li><li class="half_rhythm"><div>The median overall survival (OS) was 26 years, and the median progression-free survival (PFS) was 25 years. The 5-, 10-, and 20-year OS rates were 84%, 78% and 64%, respectively. The 5-, 10-, and 20-year PFS rates were 74%, 69%, and 64%, respectively. </div></li><li class="half_rhythm"><div>In multivariable actuarial analysis, prognostic factors associated with worse OS included poorly differentiated subtype, radiographical progression prior to radiation therapy, larger treatment volume, and lower clivus location.</div></li></ul></div></li></ol></div><div id="CDR0000800112__2470"><h3>Tyrosine Kinase Inhibitor (TKI) Therapy</h3><p id="CDR0000800112__1004">Chordomas overexpress <i>PDGFRA</i>, <i>PDGFRB</i>, and <i>KIT</i>. Because of this finding, imatinib mesylate has been studied in adults with chordomas.[<a class="bk_pop" href="#CDR0000800112_rl_1002_13">13</a>,<a class="bk_pop" href="#CDR0000800112_rl_1002_14">14</a>]</p><p id="CDR0000800112__2474">In one study, 50 adults with chordomas were treated with imatinib and evaluated by Response Evaluation Criteria In Solid Tumors (RECIST) guidelines. One patient had a partial response and 28 additional patients had stable disease at 6 months.[<a class="bk_pop" href="#CDR0000800112_rl_1002_14">14</a>] The low rate of RECIST responses and the potentially slow natural course of the disease complicate the assessment of the efficacy of imatinib for chordoma.[<a class="bk_pop" href="#CDR0000800112_rl_1002_14">14</a>]</p><p id="CDR0000800112__2471"> Other TKIs and combinations involving TKIs have been studied in adults.[<a class="bk_pop" href="#CDR0000800112_rl_1002_15">15</a>-<a class="bk_pop" href="#CDR0000800112_rl_1002_17">17</a>] </p><p id="CDR0000800112__2475">One multicenter French retrospective study reported five patients who had partial responses to treatment with either imatinib, sorafenib, or erlotinib. The median PFS was 36 months.[<a class="bk_pop" href="#CDR0000800112_rl_1002_18">18</a>]</p></div><div id="CDR0000800112__2472"><h3>Chemotherapy</h3><p id="CDR0000800112__1346">There are only a few anecdotal reports of the use of cytotoxic chemotherapy after surgery alone or surgery plus radiation therapy. Treatment with ifosfamide/etoposide and vincristine/doxorubicin/cyclophosphamide was beneficial in some reports.[<a class="bk_pop" href="#CDR0000800112_rl_1002_19">19</a>,<a class="bk_pop" href="#CDR0000800112_rl_1002_20">20</a>] The role for chemotherapy in the treatment of this disease is uncertain. </p><p id="CDR0000800112__1005">Recurrences are usually local but can include distant metastases to the lungs or bone.</p></div><div id="CDR0000800112_rl_1002"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800112_rl_1002_1">John L, Smith H, Ilanchezhian M, et al.: The NIH pediatric/young adult chordoma clinic and natural history study: Making advances in a very rare tumor. Pediatr Blood Cancer : e30358, 2023. [<a href="/pmc/articles/PMC10739575/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC10739575</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/37347686" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 37347686</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_2">Yasuda M, Bresson D, Chibbaro S, et al.: Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients. Neurosurg Rev 35 (2): 171-82; discussion 182-3, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/21863225" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21863225</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_3">DeLaney TF, Liebsch NJ, Pedlow FX, et al.: Long-term results of Phase II study of high dose photon/proton radiotherapy in the management of spine chordomas, chondrosarcomas, and other sarcomas. J Surg Oncol 110 (2): 115-22, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/24752878" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24752878</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_4">Rassi MS, Hulou MM, Almefty K, et al.: Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins' Law. Neurosurgery 82 (5): 652-660, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/28521059" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28521059</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_5">Hug EB, Sweeney RA, Nurre PM, et al.: Proton radiotherapy in management of pediatric base of skull tumors. Int J Radiat Oncol Biol Phys 52 (4): 1017-24, 2002. [<a href="https://pubmed.ncbi.nlm.nih.gov/11958897" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11958897</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_6">Noël G, Habrand JL, Jauffret E, et al.: Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine. Prognostic factors and patterns of failure. Strahlenther Onkol 179 (4): 241-8, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12707713" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12707713</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_7">Lim PS, Tran S, Kroeze SGC, et al.: Outcomes of adolescents and young adults treated for brain and skull base tumors with pencil beam scanning proton therapy. Pediatr Blood Cancer 67 (12): e28664, 2020. [<a href="https://pubmed.ncbi.nlm.nih.gov/32881313" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 32881313</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_8">Indelicato DJ, Rotondo RL, Mailhot Vega RB, et al.: Local Control After Proton Therapy for Pediatric Chordoma. Int J Radiat Oncol Biol Phys 109 (5): 1406-1413, 2021. [<a href="https://pubmed.ncbi.nlm.nih.gov/33253819" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33253819</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_9">Rombi B, Ares C, Hug EB, et al.: Spot-scanning proton radiation therapy for pediatric chordoma and chondrosarcoma: clinical outcome of 26 patients treated at paul scherrer institute. Int J Radiat Oncol Biol Phys 86 (3): 578-84, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23582853" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23582853</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_10">Rutz HP, Weber DC, Goitein G, et al.: Postoperative spot-scanning proton radiation therapy for chordoma and chondrosarcoma in children and adolescents: initial experience at paul scherrer institute. Int J Radiat Oncol Biol Phys 71 (1): 220-5, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18068310" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18068310</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_11">McDowell MM, Zwagerman NT, Wang EW, et al.: Long-term outcomes in the treatment of pediatric skull base chordomas in the endoscopic endonasal era. J Neurosurg Pediatr 27 (2): 170-179, 2020. [<a href="https://pubmed.ncbi.nlm.nih.gov/33254137" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33254137</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_12">Ioakeim-Ioannidou M, Niemierko A, Kim DW, et al.: Surgery and proton radiation therapy for pediatric base of skull chordomas: Long-term clinical outcomes for 204 patients. Neuro Oncol 25 (9): 1686-1697, 2023. [<a href="/pmc/articles/PMC10484173/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC10484173</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/37029730" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 37029730</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_13">Casali PG, Messina A, Stacchiotti S, et al.: Imatinib mesylate in chordoma. Cancer 101 (9): 2086-97, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15372471" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15372471</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_14">Stacchiotti S, Longhi A, Ferraresi V, et al.: Phase II study of imatinib in advanced chordoma. J Clin Oncol 30 (9): 914-20, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22331945" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22331945</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_15">Lindén O, Stenberg L, Kjellén E: Regression of cervical spinal cord compression in a patient with chordoma following treatment with cetuximab and gefitinib. Acta Oncol 48 (1): 158-9, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/18752082" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18752082</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_16">Singhal N, Kotasek D, Parnis FX: Response to erlotinib in a patient with treatment refractory chordoma. Anticancer Drugs 20 (10): 953-5, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19730087" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19730087</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_17">Stacchiotti S, Marrari A, Tamborini E, et al.: Response to imatinib plus sirolimus in advanced chordoma. Ann Oncol 20 (11): 1886-94, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19570961" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19570961</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_18">Lebellec L, Chauffert B, Blay JY, et al.: Advanced chordoma treated by first-line molecular targeted therapies: Outcomes and prognostic factors. A retrospective study of the French Sarcoma Group (GSF/GETO) and the Association des Neuro-Oncologues d'Expression Française (ANOCEF). Eur J Cancer 79: 119-128, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28478340" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28478340</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_19">Dhall G, Traverso M, Finlay JL, et al.: The role of chemotherapy in pediatric clival chordomas. J Neurooncol 103 (3): 657-62, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21052774" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21052774</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800112_rl_1002_20">Al-Rahawan MM, Siebert JD, Mitchell CS, et al.: Durable complete response to chemotherapy in an infant with a clival chordoma. Pediatr Blood Cancer 59 (2): 323-5, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/21922644" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21922644</span></a>]</div></li></ol></div></div><div id="CDR0000800112__2008"><h2 id="_CDR0000800112__2008_">Treatment Options Under Clinical Evaluation for Childhood Chordoma</h2><p id="CDR0000800112__2009">Information about National Cancer Institute (NCI)–supported clinical trials can be found on the <a href="https://www.cancer.gov/research/participate/clinical-trials-search" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>. For information about clinical trials sponsored by other organizations, see the <a href="https://clinicaltrials.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a>.</p><p id="CDR0000800112__2010">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000800112__2011"><li class="half_rhythm"><div><b><a href="https://www.cancer.gov/clinicaltrials/NCT05286801" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PEPN2121 (NCT05286801)</a></b> (Tiragolumab and Atezolizumab for the Treatment of Relapsed or Refractory <i>SMARCB1</i>- or <i>SMARCA4</i>-Deficient Tumors)<b>:</b> This study is evaluating the combination of a PD-L1 targeting antibody (atezolizumab) with a TIGIT targeting antibody (tiragolumab) for patients with <i>SMARCB1</i>- or <i>SMARCA4</i>-deficient tumors.</div></li></ul></div><div id="CDR0000800112__2404"><h2 id="_CDR0000800112__2404_">Latest Updates to This Summary (09/16/2024)</h2><p id="CDR0000800112__2405">The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.</p><p id="CDR0000800112__2503">This summary was comprehensively reviewed.</p><p id="CDR0000800112__disclaimerHP_3">This summary is written and maintained by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric Treatment Editorial Board</a>, which is
|
|
editorially independent of NCI. The summary reflects an independent review of
|
|
the literature and does not represent a policy statement of NCI or NIH. More
|
|
information about summary policies and the role of the PDQ Editorial Boards in
|
|
maintaining the PDQ summaries can be found on the <a href="#CDR0000800112__AboutThis_1">About This PDQ Summary</a> and <a href="https://www.cancer.gov/publications/pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ® Cancer Information for Health Professionals</a> pages.
|
|
</p></div><div id="CDR0000800112__AboutThis_1"><h2 id="_CDR0000800112__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000800112__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000800112__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric chordoma. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.</p></div><div id="CDR0000800112__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000800112__AboutThis_5">This summary is reviewed regularly and updated as necessary by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric Treatment Editorial Board</a>, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).</p><p id="CDR0000800112__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000800112__AboutThis_6"><li class="half_rhythm"><div>be discussed at a meeting,</div></li><li class="half_rhythm"><div>be cited with text, or</div></li><li class="half_rhythm"><div>replace or update an existing article that is already cited.</div></li></ul><p id="CDR0000800112__AboutThis_7">Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.</p><p>The lead reviewers for Childhood Chordoma Treatment are:</p><ul><li class="half_rhythm"><div>Denise Adams, MD (Children's Hospital Boston)</div></li><li class="half_rhythm"><div>Karen J. Marcus, MD, FACR (Dana-Farber of Boston Children's Cancer Center and Blood Disorders Harvard Medical School)</div></li><li class="half_rhythm"><div>William H. Meyer, MD</div></li><li class="half_rhythm"><div>Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)</div></li><li class="half_rhythm"><div>Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)</div></li><li class="half_rhythm"><div>Alberto S. Pappo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>D. Williams Parsons, MD, PhD (Texas Children's Hospital)</div></li><li class="half_rhythm"><div>Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)</div></li><li class="half_rhythm"><div>Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>Stephen J. Shochat, MD (St. Jude Children's Research Hospital)</div></li></ul><p id="CDR0000800112__AboutThis_9">Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.</p></div><div id="CDR0000800112__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000800112__AboutThis_11">Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a <a href="/books/n/pdqcis/CDR0000062796/">formal evidence ranking system</a> in developing its level-of-evidence designations.</p></div><div id="CDR0000800112__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000800112__AboutThis_13">PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”</p><p id="CDR0000800112__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000800112__AboutThis_15">PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Chordoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/bone/hp/child-chordoma-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/bone/hp/child-chordoma-treatment-pdq</a>. Accessed <MM/DD/YYYY>. [PMID: 31909945]</p><p id="CDR0000800112__AboutThis_16">Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in <a href="https://visualsonline.cancer.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Visuals Online</a>, a collection of over 2,000 scientific images.
|
|
</p></div><div id="CDR0000800112__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000800112__AboutThis_18">Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the <a href="https://www.cancer.gov/about-cancer/managing-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Managing Cancer Care</a> page.</p></div><div id="CDR0000800112__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000800112__AboutThis_21">More information about contacting us or receiving help with the Cancer.gov website can be found on our <a href="https://www.cancer.gov/contact" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Contact Us for Help</a> page. Questions can also be submitted to Cancer.gov through the website’s <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>.</p></div></div></div></div>
|
|
<div class="post-content"><div><div class="half_rhythm"><a href="/books/about/copyright/">Copyright Notice</a></div><div class="small"><span class="label">Bookshelf ID: NBK552289</span><span class="label">PMID: <a href="https://pubmed.ncbi.nlm.nih.gov/31909945" title="PubMed record of this page" ref="pagearea=meta&targetsite=entrez&targetcat=link&targettype=pubmed">31909945</a></span></div></div></div>
|
|
|
|
</div>
|
|
|
|
<!-- Custom content below content -->
|
|
<div class="col4">
|
|
|
|
</div>
|
|
|
|
|
|
<!-- Book content -->
|
|
|
|
<!-- Custom contetnt below bottom nav -->
|
|
<div class="col5">
|
|
|
|
</div>
|
|
</div>
|
|
|
|
<div id="rightcolumn" class="four_col col last">
|
|
<!-- Custom content above discovery portlets -->
|
|
<div class="col6">
|
|
<div id="ncbi_share_book"><a href="#" class="ncbi_share" data-ncbi_share_config="popup:false,shorten:true" ref="id=NBK552289&db=books">Share</a></div>
|
|
|
|
</div>
|
|
<div xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance"></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Views</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="PDF_download" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="/books/NBK552289.13/?report=reader">PubReader</a></li><li><a href="/books/NBK552289.13/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK552289" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK552289" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Pediatric Treatment Editorial Board. Childhood Chordoma Treatment (PDQ®): Health Professional Version. 2024 Sep 16. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. <span class="bk_cite_avail"></span></div></div></li><li><a href="#" class="toggle-glossary-link" title="Enable/disable links to the glossary">Disable Glossary Links</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Version History</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter shutter_closed" title="Show/hide content" remembercollapsed="true" pgsec_name="version_history" id="Shutter"></a></div><div class="portlet_content" style="display: none;"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><span class="bk_col_itm"><a href="/books/NBK552289.14/">NBK552289.14</a></span> November 29, 2024</li><li><span class="bk_col_itm">NBK552289.13</span> September 16, 2024 (Displayed Version)</li><li><span class="bk_col_itm"><a href="/books/NBK552289.12/">NBK552289.12</a></span> April 11, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK552289.11/">NBK552289.11</a></span> December 15, 2023</li><li><span class="bk_col_itm"><a href="/books/NBK552289.10/">NBK552289.10</a></span> January 5, 2023</li><li><span class="bk_col_itm"><a href="/books/NBK552289.9/">NBK552289.9</a></span> December 9, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK552289.8/">NBK552289.8</a></span> April 19, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK552289.7/">NBK552289.7</a></span> February 23, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK552289.6/">NBK552289.6</a></span> December 17, 2021</li><li><span class="bk_col_itm"><a href="/books/NBK552289.5/">NBK552289.5</a></span> October 5, 2021</li><li><span class="bk_col_itm"><a href="/books/NBK552289.4/">NBK552289.4</a></span> February 11, 2021</li><li><span class="bk_col_itm"><a href="/books/NBK552289.3/">NBK552289.3</a></span> December 7, 2020</li><li><span class="bk_col_itm"><a href="/books/NBK552289.2/">NBK552289.2</a></span> January 31, 2020</li><li><span class="bk_col_itm"><a href="/books/NBK552289.1/">NBK552289.1</a></span> December 23, 2019</li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>In this Page</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="page-toc" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="#CDR0000800112__996" ref="log$=inpage&link_id=inpage">Incidence</a></li><li><a href="#CDR0000800112__1000" ref="log$=inpage&link_id=inpage">Clinical Presentation and Diagnosis</a></li><li><a href="#CDR0000800112__998" ref="log$=inpage&link_id=inpage">Prognosis and Molecular Features</a></li><li><a href="#CDR0000800112__1864" ref="log$=inpage&link_id=inpage">Special Considerations for the Treatment of Children With Cancer</a></li><li><a href="#CDR0000800112__1002" ref="log$=inpage&link_id=inpage">Treatment of Childhood Chordoma</a></li><li><a href="#CDR0000800112__2008" ref="log$=inpage&link_id=inpage">Treatment Options Under Clinical Evaluation for Childhood Chordoma</a></li><li><a href="#CDR0000800112__2404" ref="log$=inpage&link_id=inpage">Latest Updates to This Summary (09/16/2024)</a></li><li><a href="#CDR0000800112__AboutThis_1" ref="log$=inpage&link_id=inpage">About This PDQ Summary</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related publications</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="document-links" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="/books/NBK552283/">Patient Version</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related information</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="discovery_db_links" id="Shutter"></a></div><div class="portlet_content"><ul><li class="brieflinkpopper"><a class="brieflinkpopperctrl" href="/books/?Db=pmc&DbFrom=books&Cmd=Link&LinkName=books_pmc_refs&IdsFromResult=4911190" ref="log$=recordlinks">PMC</a><div class="brieflinkpop offscreen_noflow">PubMed Central citations</div></li><li class="brieflinkpopper"><a class="brieflinkpopperctrl" href="/books/?Db=pubmed&DbFrom=books&Cmd=Link&LinkName=books_pubmed_refs&IdsFromResult=4911190" ref="log$=recordlinks">PubMed</a><div class="brieflinkpop offscreen_noflow">Links to PubMed</div></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Similar articles in PubMed</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="PBooksDiscovery_RA" id="Shutter"></a></div><div class="portlet_content"><ul><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/31909946" ref="ordinalpos=1&linkpos=1&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Melanoma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Melanoma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389243" ref="ordinalpos=1&linkpos=2&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/31593397" ref="ordinalpos=1&linkpos=3&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Mesothelioma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Mesothelioma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/31909942" ref="ordinalpos=1&linkpos=4&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Pheochromocytoma and Paraganglioma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Pheochromocytoma and Paraganglioma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/29337483" ref="ordinalpos=1&linkpos=5&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Esthesioneuroblastoma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Esthesioneuroblastoma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li></ul><a class="seemore" href="/sites/entrez?db=pubmed&cmd=link&linkname=pubmed_pubmed_reviews&uid=31909945" ref="ordinalpos=1&log$=relatedreviews_seeall&logdbfrom=pubmed">See reviews...</a><a class="seemore" href="/sites/entrez?db=pubmed&cmd=link&linkname=pubmed_pubmed&uid=31909945" ref="ordinalpos=1&log$=relatedarticles_seeall&logdbfrom=pubmed">See all...</a></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Recent Activity</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="recent_activity" id="Shutter"></a></div><div class="portlet_content"><div xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" id="HTDisplay" class=""><div class="action"><a href="javascript:historyDisplayState('ClearHT')">Clear</a><a href="javascript:historyDisplayState('HTOff')" class="HTOn">Turn Off</a><a href="javascript:historyDisplayState('HTOn')" class="HTOff">Turn On</a></div><ul id="activity"><li class="ra_rcd ralinkpopper two_line"><a class="htb ralinkpopperctrl" ref="log$=activity&linkpos=1" href="/portal/utils/pageresolver.fcgi?recordid=67c98361ab82281a809dce8e">Childhood Chordoma Treatment (PDQ®) - PDQ Cancer Information Summaries</a><div class="ralinkpop offscreen_noflow">Childhood Chordoma Treatment (PDQ®) - PDQ Cancer Information Summaries<div class="brieflinkpopdesc"></div></div><div class="tertiary"></div></li><li class="ra_qry two_line"><a class="htb" ref="log$=activity&linkpos=2" href="/portal/utils/pageresolver.fcgi?recordid=67c98357f4a390645e97249a">PMC Links for Books (Select 5540604) <span class="number">(7)</span></a><div class="tertiary">PMC</div></li><li class="ra_rcd ralinkpopper two_line"><a class="htb ralinkpopperctrl" ref="log$=activity&linkpos=3" href="/portal/utils/pageresolver.fcgi?recordid=67c98356b15b832ebc6c58e4">RUNX1-Familial Platelet Disorder (PDQ®) - PDQ Cancer Information Summaries</a><div class="ralinkpop offscreen_noflow">RUNX1-Familial Platelet Disorder (PDQ®) - PDQ Cancer Information Summaries<div class="brieflinkpopdesc"></div></div><div class="tertiary"></div></li><li class="ra_qry two_line"><a class="htb" ref="log$=activity&linkpos=4" href="/portal/utils/pageresolver.fcgi?recordid=67c9834dab82281a809d422f">PMC Links for Books (Select 2828458) <span class="number">(113)</span></a><div class="tertiary">PMC</div></li><li class="ra_rcd ralinkpopper two_line"><a class="htb ralinkpopperctrl" ref="log$=activity&linkpos=5" href="/portal/utils/pageresolver.fcgi?recordid=67c9834cab82281a809d3a10">Childhood Non-Hodgkin Lymphoma Treatment (PDQ®) - PDQ Cancer Information Summari...</a><div class="ralinkpop offscreen_noflow">Childhood Non-Hodgkin Lymphoma Treatment (PDQ®) - PDQ Cancer Information Summaries<div class="brieflinkpopdesc"></div></div><div class="tertiary"></div></li></ul><p class="HTOn">Your browsing activity is empty.</p><p class="HTOff">Activity recording is turned off.</p><p id="turnOn" class="HTOff"><a href="javascript:historyDisplayState('HTOn')">Turn recording back on</a></p><a class="seemore" href="/sites/myncbi/recentactivity">See more...</a></div></div></div>
|
|
|
|
<!-- Custom content below discovery portlets -->
|
|
<div class="col7">
|
|
|
|
</div>
|
|
</div>
|
|
</div>
|
|
|
|
<!-- Custom content after all -->
|
|
<div class="col8">
|
|
|
|
</div>
|
|
<div class="col9">
|
|
|
|
</div>
|
|
|
|
<script type="text/javascript" src="/corehtml/pmc/js/jquery.scrollTo-1.4.2.js"></script>
|
|
<script type="text/javascript">
|
|
(function($){
|
|
$('.skiplink').each(function(i, item){
|
|
var href = $($(item).attr('href'));
|
|
href.attr('tabindex', '-1').addClass('skiptarget'); // ensure the target can receive focus
|
|
$(item).on('click', function(event){
|
|
event.preventDefault();
|
|
$.scrollTo(href, 0, {
|
|
onAfter: function(){
|
|
href.focus();
|
|
}
|
|
});
|
|
});
|
|
});
|
|
})(jQuery);
|
|
</script>
|
|
</div>
|
|
<div class="bottom">
|
|
|
|
<div id="NCBIFooter_dynamic">
|
|
<!--<component id="Breadcrumbs" label="breadcrumbs"/>
|
|
<component id="Breadcrumbs" label="helpdesk"/>-->
|
|
|
|
</div>
|
|
|
|
<div class="footer" id="footer">
|
|
<section class="icon-section">
|
|
<div id="icon-section-header" class="icon-section_header">Follow NCBI</div>
|
|
<div class="grid-container container">
|
|
<div class="icon-section_container">
|
|
<a class="footer-icon" id="footer_twitter" href="https://twitter.com/ncbi" aria-label="Twitter"><svg xmlns="http://www.w3.org/2000/svg" data-name="Layer 1" viewBox="0 0 300 300">
|
|
<defs>
|
|
<style>
|
|
.cls-11 {
|
|
fill: #737373;
|
|
}
|
|
</style>
|
|
</defs>
|
|
<title>Twitter</title>
|
|
<path class="cls-11" d="M250.11,105.48c-7,3.14-13,3.25-19.27.14,8.12-4.86,8.49-8.27,11.43-17.46a78.8,78.8,0,0,1-25,9.55,39.35,39.35,0,0,0-67,35.85,111.6,111.6,0,0,1-81-41.08A39.37,39.37,0,0,0,81.47,145a39.08,39.08,0,0,1-17.8-4.92c0,.17,0,.33,0,.5a39.32,39.32,0,0,0,31.53,38.54,39.26,39.26,0,0,1-17.75.68,39.37,39.37,0,0,0,36.72,27.3A79.07,79.07,0,0,1,56,223.34,111.31,111.31,0,0,0,116.22,241c72.3,0,111.83-59.9,111.83-111.84,0-1.71,0-3.4-.1-5.09C235.62,118.54,244.84,113.37,250.11,105.48Z">
|
|
</path>
|
|
</svg></a>
|
|
<a class="footer-icon" id="footer_facebook" href="https://www.facebook.com/ncbi.nlm" aria-label="Facebook"><svg xmlns="http://www.w3.org/2000/svg" data-name="Layer 1" viewBox="0 0 300 300">
|
|
<title>Facebook</title>
|
|
<path class="cls-11" d="M210.5,115.12H171.74V97.82c0-8.14,5.39-10,9.19-10h27.14V52l-39.32-.12c-35.66,0-42.42,26.68-42.42,43.77v19.48H99.09v36.32h27.24v109h45.41v-109h35Z">
|
|
</path>
|
|
</svg></a>
|
|
<a class="footer-icon" id="footer_linkedin" href="https://www.linkedin.com/company/ncbinlm" aria-label="LinkedIn"><svg xmlns="http://www.w3.org/2000/svg" data-name="Layer 1" viewBox="0 0 300 300">
|
|
<title>LinkedIn</title>
|
|
<path class="cls-11" d="M101.64,243.37H57.79v-114h43.85Zm-22-131.54h-.26c-13.25,0-21.82-10.36-21.82-21.76,0-11.65,8.84-21.15,22.33-21.15S101.7,78.72,102,90.38C102,101.77,93.4,111.83,79.63,111.83Zm100.93,52.61A17.54,17.54,0,0,0,163,182v61.39H119.18s.51-105.23,0-114H163v13a54.33,54.33,0,0,1,34.54-12.66c26,0,44.39,18.8,44.39,55.29v58.35H198.1V182A17.54,17.54,0,0,0,180.56,164.44Z">
|
|
</path>
|
|
</svg></a>
|
|
<a class="footer-icon" id="footer_github" href="https://github.com/ncbi" aria-label="GitHub"><svg xmlns="http://www.w3.org/2000/svg" data-name="Layer 1" viewBox="0 0 300 300">
|
|
<defs>
|
|
<style>
|
|
.cls-11,
|
|
.cls-12 {
|
|
fill: #737373;
|
|
}
|
|
|
|
.cls-11 {
|
|
fill-rule: evenodd;
|
|
}
|
|
</style>
|
|
</defs>
|
|
<title>GitHub</title>
|
|
<path class="cls-11" d="M151.36,47.28a105.76,105.76,0,0,0-33.43,206.1c5.28,1,7.22-2.3,7.22-5.09,0-2.52-.09-10.85-.14-19.69-29.42,6.4-35.63-12.48-35.63-12.48-4.81-12.22-11.74-15.47-11.74-15.47-9.59-6.56.73-6.43.73-6.43,10.61.75,16.21,10.9,16.21,10.9,9.43,16.17,24.73,11.49,30.77,8.79,1-6.83,3.69-11.5,6.71-14.14C108.57,197.1,83.88,188,83.88,147.51a40.92,40.92,0,0,1,10.9-28.39c-1.1-2.66-4.72-13.42,1-28,0,0,8.88-2.84,29.09,10.84a100.26,100.26,0,0,1,53,0C198,88.3,206.9,91.14,206.9,91.14c5.76,14.56,2.14,25.32,1,28a40.87,40.87,0,0,1,10.89,28.39c0,40.62-24.74,49.56-48.29,52.18,3.79,3.28,7.17,9.71,7.17,19.58,0,14.15-.12,25.54-.12,29,0,2.82,1.9,6.11,7.26,5.07A105.76,105.76,0,0,0,151.36,47.28Z">
|
|
</path>
|
|
<path class="cls-12" d="M85.66,199.12c-.23.52-1.06.68-1.81.32s-1.2-1.06-.95-1.59,1.06-.69,1.82-.33,1.21,1.07.94,1.6Zm-1.3-1">
|
|
</path>
|
|
<path class="cls-12" d="M90,203.89c-.51.47-1.49.25-2.16-.49a1.61,1.61,0,0,1-.31-2.19c.52-.47,1.47-.25,2.17.49s.82,1.72.3,2.19Zm-1-1.08">
|
|
</path>
|
|
<path class="cls-12" d="M94.12,210c-.65.46-1.71,0-2.37-.91s-.64-2.07,0-2.52,1.7,0,2.36.89.65,2.08,0,2.54Zm0,0"></path>
|
|
<path class="cls-12" d="M99.83,215.87c-.58.64-1.82.47-2.72-.41s-1.18-2.06-.6-2.7,1.83-.46,2.74.41,1.2,2.07.58,2.7Zm0,0">
|
|
</path>
|
|
<path class="cls-12" d="M107.71,219.29c-.26.82-1.45,1.2-2.64.85s-2-1.34-1.74-2.17,1.44-1.23,2.65-.85,2,1.32,1.73,2.17Zm0,0">
|
|
</path>
|
|
<path class="cls-12" d="M116.36,219.92c0,.87-1,1.59-2.24,1.61s-2.29-.68-2.3-1.54,1-1.59,2.26-1.61,2.28.67,2.28,1.54Zm0,0">
|
|
</path>
|
|
<path class="cls-12" d="M124.42,218.55c.15.85-.73,1.72-2,1.95s-2.37-.3-2.52-1.14.73-1.75,2-2,2.37.29,2.53,1.16Zm0,0"></path>
|
|
</svg></a>
|
|
<a class="footer-icon" id="footer_blog" href="https://ncbiinsights.ncbi.nlm.nih.gov/" aria-label="Blog">
|
|
<svg xmlns="http://www.w3.org/2000/svg" id="Layer_1" data-name="Layer 1" viewBox="0 0 40 40">
|
|
<defs><style>.cls-1{fill:#737373;}</style></defs>
|
|
<title>NCBI Insights Blog</title>
|
|
<path class="cls-1" d="M14,30a4,4,0,1,1-4-4,4,4,0,0,1,4,4Zm11,3A19,19,0,0,0,7.05,15a1,1,0,0,0-1,1v3a1,1,0,0,0,.93,1A14,14,0,0,1,20,33.07,1,1,0,0,0,21,34h3a1,1,0,0,0,1-1Zm9,0A28,28,0,0,0,7,6,1,1,0,0,0,6,7v3a1,1,0,0,0,1,1A23,23,0,0,1,29,33a1,1,0,0,0,1,1h3A1,1,0,0,0,34,33Z"></path>
|
|
</svg>
|
|
</a>
|
|
</div>
|
|
</div>
|
|
</section>
|
|
|
|
<section class="container-fluid bg-primary">
|
|
<div class="container pt-5">
|
|
<div class="row mt-3">
|
|
<div class="col-lg-3 col-12">
|
|
<p><a class="text-white" href="https://www.nlm.nih.gov/socialmedia/index.html">Connect with NLM</a></p>
|
|
<ul class="list-inline social_media">
|
|
<li class="list-inline-item"><a href="https://twitter.com/NLM_NIH" aria-label="Twitter" target="_blank" rel="noopener noreferrer"><svg xmlns="http://www.w3.org/2000/svg" xmlns:xlink="http://www.w3.org/1999/xlink" version="1.1" x="0px" y="0px" viewBox="0 0 249 249" style="enable-background:new 0 0 249 249;" xml:space="preserve">
|
|
<style type="text/css">
|
|
.st20 {
|
|
fill: #FFFFFF;
|
|
}
|
|
|
|
.st30 {
|
|
fill: none;
|
|
stroke: #FFFFFF;
|
|
stroke-width: 8;
|
|
stroke-miterlimit: 10;
|
|
}
|
|
</style>
|
|
<title>Twitter</title>
|
|
<g>
|
|
<g>
|
|
<g>
|
|
<path class="st20" d="M192.9,88.1c-5,2.2-9.2,2.3-13.6,0.1c5.7-3.4,6-5.8,8.1-12.3c-5.4,3.2-11.4,5.5-17.6,6.7 c-10.5-11.2-28.1-11.7-39.2-1.2c-7.2,6.8-10.2,16.9-8,26.5c-22.3-1.1-43.1-11.7-57.2-29C58,91.6,61.8,107.9,74,116 c-4.4-0.1-8.7-1.3-12.6-3.4c0,0.1,0,0.2,0,0.4c0,13.2,9.3,24.6,22.3,27.2c-4.1,1.1-8.4,1.3-12.5,0.5c3.6,11.3,14,19,25.9,19.3 c-11.6,9.1-26.4,13.2-41.1,11.5c12.7,8.1,27.4,12.5,42.5,12.5c51,0,78.9-42.2,78.9-78.9c0-1.2,0-2.4-0.1-3.6 C182.7,97.4,189.2,93.7,192.9,88.1z"></path>
|
|
</g>
|
|
</g>
|
|
<circle class="st30" cx="124.4" cy="128.8" r="108.2"></circle>
|
|
</g>
|
|
</svg></a></li>
|
|
<li class="list-inline-item"><a href="https://www.facebook.com/nationallibraryofmedicine" aria-label="Facebook" rel="noopener noreferrer" target="_blank">
|
|
<svg xmlns="http://www.w3.org/2000/svg" xmlns:xlink="http://www.w3.org/1999/xlink" version="1.1" x="0px" y="0px" viewBox="0 0 249 249" style="enable-background:new 0 0 249 249;" xml:space="preserve">
|
|
<style type="text/css">
|
|
.st10 {
|
|
fill: #FFFFFF;
|
|
}
|
|
|
|
.st110 {
|
|
fill: none;
|
|
stroke: #FFFFFF;
|
|
stroke-width: 8;
|
|
stroke-miterlimit: 10;
|
|
}
|
|
</style>
|
|
<title>Facebook</title>
|
|
<g>
|
|
<g>
|
|
<path class="st10" d="M159,99.1h-24V88.4c0-5,3.3-6.2,5.7-6.2h16.8V60l-24.4-0.1c-22.1,0-26.2,16.5-26.2,27.1v12.1H90v22.5h16.9 v67.5H135v-67.5h21.7L159,99.1z"></path>
|
|
</g>
|
|
</g>
|
|
<circle class="st110" cx="123.6" cy="123.2" r="108.2"></circle>
|
|
</svg>
|
|
</a></li>
|
|
<li class="list-inline-item"><a href="https://www.youtube.com/user/NLMNIH" aria-label="Youtube" target="_blank" rel="noopener noreferrer"><svg xmlns="http://www.w3.org/2000/svg" xmlns:xlink="http://www.w3.org/1999/xlink" version="1.1" x="0px" y="0px" viewBox="0 0 249 249" style="enable-background:new 0 0 249 249;" xml:space="preserve">
|
|
<title>Youtube</title>
|
|
<style type="text/css">
|
|
.st4 {
|
|
fill: none;
|
|
stroke: #FFFFFF;
|
|
stroke-width: 8;
|
|
stroke-miterlimit: 10;
|
|
}
|
|
|
|
.st5 {
|
|
fill: #FFFFFF;
|
|
}
|
|
</style>
|
|
<circle class="st4" cx="124.2" cy="123.4" r="108.2"></circle>
|
|
<g transform="translate(0,-952.36218)">
|
|
<path class="st5" d="M88.4,1037.4c-10.4,0-18.7,8.3-18.7,18.7v40.1c0,10.4,8.3,18.7,18.7,18.7h72.1c10.4,0,18.7-8.3,18.7-18.7 v-40.1c0-10.4-8.3-18.7-18.7-18.7H88.4z M115.2,1058.8l29.4,17.4l-29.4,17.4V1058.8z"></path>
|
|
</g>
|
|
</svg></a></li>
|
|
</ul>
|
|
</div>
|
|
<div class="col-lg-3 col-12">
|
|
<p class="address_footer text-white">National Library of Medicine<br />
|
|
<a href="https://www.google.com/maps/place/8600+Rockville+Pike,+Bethesda,+MD+20894/@38.9959508,-77.101021,17z/data=!3m1!4b1!4m5!3m4!1s0x89b7c95e25765ddb:0x19156f88b27635b8!8m2!3d38.9959508!4d-77.0988323" class="text-white" target="_blank" rel="noopener noreferrer">8600 Rockville Pike<br />
|
|
Bethesda, MD 20894</a></p>
|
|
</div>
|
|
<div class="col-lg-3 col-12 centered-lg">
|
|
<p><a href="https://www.nlm.nih.gov/web_policies.html" class="text-white">Web Policies</a><br />
|
|
<a href="https://www.nih.gov/institutes-nih/nih-office-director/office-communications-public-liaison/freedom-information-act-office" class="text-white">FOIA</a><br />
|
|
<a href="https://www.hhs.gov/vulnerability-disclosure-policy/index.html" class="text-white" id="vdp">HHS Vulnerability Disclosure</a></p>
|
|
</div>
|
|
<div class="col-lg-3 col-12 centered-lg">
|
|
<p><a class="supportLink text-white" href="https://support.nlm.nih.gov/">Help</a><br />
|
|
<a href="https://www.nlm.nih.gov/accessibility.html" class="text-white">Accessibility</a><br />
|
|
<a href="https://www.nlm.nih.gov/careers/careers.html" class="text-white">Careers</a></p>
|
|
</div>
|
|
</div>
|
|
<div class="row">
|
|
<div class="col-lg-12 centered-lg">
|
|
<nav class="bottom-links">
|
|
<ul class="mt-3">
|
|
<li>
|
|
<a class="text-white" href="//www.nlm.nih.gov/">NLM</a>
|
|
</li>
|
|
<li>
|
|
<a class="text-white" href="https://www.nih.gov/">NIH</a>
|
|
</li>
|
|
<li>
|
|
<a class="text-white" href="https://www.hhs.gov/">HHS</a>
|
|
</li>
|
|
<li>
|
|
<a class="text-white" href="https://www.usa.gov/">USA.gov</a>
|
|
</li>
|
|
</ul>
|
|
</nav>
|
|
</div>
|
|
</div>
|
|
</div>
|
|
</section>
|
|
<script type="text/javascript" src="/portal/portal3rc.fcgi/rlib/js/InstrumentOmnitureBaseJS/InstrumentNCBIConfigJS/InstrumentNCBIBaseJS/InstrumentPageStarterJS.js?v=1"> </script>
|
|
<script type="text/javascript" src="/portal/portal3rc.fcgi/static/js/hfjs2.js"> </script>
|
|
</div>
|
|
</div>
|
|
</div>
|
|
<!--/.page-->
|
|
</div>
|
|
<!--/.wrap-->
|
|
</div><!-- /.twelve_col -->
|
|
</div>
|
|
<!-- /.grid -->
|
|
|
|
<span class="PAFAppResources"></span>
|
|
|
|
<!-- BESelector tab -->
|
|
|
|
|
|
|
|
<noscript><img alt="statistics" src="/stat?jsdisabled=true&ncbi_db=books&ncbi_pdid=book-part&ncbi_acc=NBK552289&ncbi_domain=pdqcis&ncbi_report=record&ncbi_type=fulltext&ncbi_objectid=&ncbi_pcid=/NBK552289.13/&ncbi_pagename=Childhood Chordoma Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf&ncbi_bookparttype=chapter&ncbi_app=bookshelf" /></noscript>
|
|
|
|
|
|
<!-- usually for JS scripts at page bottom -->
|
|
<!--<component id="PageFixtures" label="styles"></component>-->
|
|
|
|
|
|
<!-- CE8B5AF87C7FFCB1_0191SID /projects/books/PBooks@9.11 portal104 v4.1.r689238 Tue, Oct 22 2024 16:10:51 -->
|
|
<span id="portal-csrf-token" style="display:none" data-token="CE8B5AF87C7FFCB1_0191SID"></span>
|
|
|
|
<script type="text/javascript" src="//static.pubmed.gov/portal/portal3rc.fcgi/4216699/js/3879255/4121861/3501987/4008961/3893018/3821238/4062932/4209313/4212053/4076480/3921943/3400083/3426610.js" snapshot="books"></script></body>
|
|
</html> |