531 lines
No EOL
118 KiB
HTML
531 lines
No EOL
118 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="NBK552292" /><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="/NBK552292.10/" /><meta name="ncbi_pagename" content="Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf" /><meta name="ncbi_bookparttype" content="chapter" /><meta name="ncbi_app" content="bookshelf" />
|
|
<!-- Logger end -->
|
|
|
|
<title>Childhood Multiple Endocrine Neoplasia (MEN) Syndromes 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 Multiple Endocrine Neoplasia (MEN) Syndromes 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="31909948" /><meta name="citation_fulltext_html_url" content="https://www.ncbi.nlm.nih.gov/books/NBK552292/" /><meta name="citation_keywords" content="multiple endocrine neoplasia" /><meta name="citation_keywords" content="Carney Complex" /><meta name="citation_keywords" content="Multiple endocrine neoplasia" /><meta name="citation_keywords" content="pediatric MEN1 syndrome" /><meta name="citation_keywords" content="pediatric MEN2A syndrome" /><meta name="citation_keywords" content="pediatric MEN2B syndrome" /><link rel="schema.DC" href="http://purl.org/DC/elements/1.0/" /><meta name="DC.Title" content="Childhood Multiple Endocrine Neoplasia (MEN) Syndromes 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/NBK552292/" /><meta name="description" content="This evidence-based, expert-reviewed summary discusses the clinical presentation, diagnosis, molecular features, and treatment of pediatric multiple endocrine neoplasia (MEN) syndromes." /><meta name="og:title" content="Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment (PDQ®)" /><meta name="og:type" content="book" /><meta name="og:description" content="This evidence-based, expert-reviewed summary discusses the clinical presentation, diagnosis, molecular features, and treatment of pediatric multiple endocrine neoplasia (MEN) syndromes." /><meta name="og:url" content="https://www.ncbi.nlm.nih.gov/books/NBK552292/" /><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/CDR0000800107/" /><link rel="canonical" href="https://www.ncbi.nlm.nih.gov/books/NBK552292/" /><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="CE8D96B37C98ED710000000000710058.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="#__NBK552292_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK552292_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="_NBK552292_"><span class="title" itemprop="name">Childhood Multiple Endocrine Neoplasia (MEN) Syndromes 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: January 3, 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="CDR0000800107__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric multiple endocrine neoplasia (MEN) syndromes. 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="CDR0000800107__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="CDR0000800107__2333"><h2 id="_CDR0000800107__2333_">General Information About Childhood Multiple Endocrine Neoplasia (MEN) Syndromes</h2><p id="CDR0000800107__85">MEN syndromes are familial disorders characterized by neoplastic
|
|
changes that affect multiple endocrine organs.[<a class="bk_pop" href="#CDR0000800107_rl_2333_1">1</a>] Changes may include hyperplasia,
|
|
benign adenomas, and carcinomas. </p><p id="CDR0000800107__1518">There are two main types of MEN syndromes:</p><ul id="CDR0000800107__1297"><li class="half_rhythm"><div>Type 1.</div></li><li class="half_rhythm"><div>Type 2, which includes the following two subtypes: <dl id="CDR0000800107__1298" class="temp-labeled-list"><dt>-</dt><dd><p class="no_top_margin">Type 2A (includes familial medullary thyroid carcinoma).</p></dd><dt>-</dt><dd><p class="no_top_margin">Type 2B.</p></dd></dl></div></li></ul><p id="CDR0000800107__1299">For more information about MEN syndromes, see <a href="/books/n/pdqcis/CDR0000062890/">Genetics of Endocrine and Neuroendocrine Neoplasias</a>.</p><div id="CDR0000800107_rl_2333"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800107_rl_2333_1">de Krijger RR: Endocrine tumor syndromes in infancy and childhood. Endocr Pathol 15 (3): 223-6, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15640547" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15640547</span></a>]</div></li></ol></div></div><div id="CDR0000800107__676"><h2 id="_CDR0000800107__676_">Clinical Presentation, Molecular Features, and Diagnostic Evaluation</h2><p id="CDR0000800107__677">The main clinical features and genetic alterations of the multiple endocrine neoplasia (MEN) syndromes are shown in <a class="figpopup" href="/books/NBK552292.10/table/CDR0000800107__678/?report=objectonly" target="object" rid-figpopup="figCDR0000800107678" rid-ob="figobCDR0000800107678">Table 1</a>.</p><div id="CDR0000800107__678" class="table"><h3><span class="title">Table 1. Multiple Endocrine Neoplasia (MEN) Syndromes With Associated Clinical Features and Genetic Alterations </span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK552292.10/table/CDR0000800107__678/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000800107__678_lrgtbl__"><table class="no_top_margin"><thead><tr><th colspan="1" rowspan="1" style="vertical-align:top;">Syndrome</th><th colspan="2" rowspan="1" style="vertical-align:top;">Clinical Features/Tumors</th><th colspan="1" rowspan="1" style="vertical-align:top;">Genetic Alterations</th></tr></thead><tbody><tr><td colspan="1" rowspan="13" style="vertical-align:top;"><b>MEN type 1 (Wermer syndrome)</b> [<a class="bk_pop" href="#CDR0000800107_rl_676_1">1</a>]</td><td colspan="2" rowspan="1" style="vertical-align:top;">
|
|
<b>Parathyroid</b>
|
|
</td><td colspan="1" rowspan="1" style="vertical-align:top;">11q13 (<i>MEN1 </i>gene)</td></tr><tr><td colspan="1" rowspan="4" style="vertical-align:top;">
|
|
<b>Pancreatic islets: </b>
|
|
</td><td colspan="1" rowspan="1" style="vertical-align:top;">Gastrinoma</td><td colspan="1" rowspan="4" style="vertical-align:top;">11q13 (<i>MEN1 </i>gene)</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Insulinoma</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Glucagonoma</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">VIPoma</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">
|
|
<b>Pituitary:</b>
|
|
</td><td colspan="1" rowspan="1" style="vertical-align:top;">Prolactinoma</td><td colspan="1" rowspan="3" style="vertical-align:top;">11q13 (<i>MEN1 </i>gene)</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Somatotropinoma</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Corticotropinoma</td></tr><tr><td colspan="1" rowspan="5" style="vertical-align:top;">
|
|
<b>Other associated tumors (less common): </b>
|
|
</td><td colspan="1" rowspan="1" style="vertical-align:top;">Carcinoid—bronchial and thymic</td><td colspan="1" rowspan="5" style="vertical-align:top;">11q13 (<i>MEN1 </i>gene)</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Adrenocortical</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Lipoma</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Angiofibroma</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Collagenoma</td></tr><tr><td colspan="1" rowspan="3" style="vertical-align:top;">
|
|
<b>MEN type 2A (Sipple syndrome)</b>
|
|
</td><td colspan="2" rowspan="1" style="vertical-align:top;">
|
|
<b>Medullary thyroid carcinoma</b>
|
|
</td><td colspan="1" rowspan="3" style="vertical-align:top;">10q11.2 (<i>RET </i>gene)</td></tr><tr><td colspan="2" rowspan="1" style="vertical-align:top;">
|
|
<b>Pheochromocytoma</b>
|
|
</td></tr><tr><td colspan="2" rowspan="1" style="vertical-align:top;">
|
|
<b>Parathyroid gland</b>
|
|
</td></tr><tr><td colspan="1" rowspan="5" style="vertical-align:top;">
|
|
<b>MEN type 2B</b>
|
|
</td><td colspan="2" rowspan="1" style="vertical-align:top;">
|
|
<b>Medullary thyroid carcinoma</b>
|
|
</td><td colspan="1" rowspan="5" style="vertical-align:top;">10q11.2 (<i>RET </i>gene)</td></tr><tr><td colspan="2" rowspan="1" style="vertical-align:top;">
|
|
<b>Pheochromocytoma</b>
|
|
</td></tr><tr><td colspan="2" rowspan="1" style="vertical-align:top;">
|
|
<b>Mucosal neuromas</b>
|
|
</td></tr><tr><td colspan="2" rowspan="1" style="vertical-align:top;">
|
|
<b>Intestinal ganglioneuromatosis</b>
|
|
</td></tr><tr><td colspan="2" rowspan="1" style="vertical-align:top;">
|
|
<b>Marfanoid habitus</b>
|
|
</td></tr></tbody></table></div></div><div id="CDR0000800107__2471"><h3>MEN Type 1 (MEN1) Syndrome (Wermer Syndrome)</h3><p id="CDR0000800107__2472">MEN1 syndrome is an autosomal dominant disorder characterized by the presence of tumors in the parathyroid, pancreatic islet cells, and anterior pituitary. Diagnosis of this syndrome should be considered when two endocrine tumors listed in <a class="figpopup" href="/books/NBK552292.10/table/CDR0000800107__678/?report=objectonly" target="object" rid-figpopup="figCDR0000800107678" rid-ob="figobCDR0000800107678">Table 1</a> are present. </p><p id="CDR0000800107__1443">Clinical practice guidelines recommend that screening for patients with MEN1 syndrome begins by the age of 5 years and continues throughout life. The tests for screening are age specific and may include yearly serum calcium, parathyroid hormone, gastrin, glucagon, secretin, proinsulin, chromogranin A, prolactin, and IGF-1. Radiological screening should include magnetic resonance imaging of the brain and computed tomography of the abdomen every 1 to 3 years.[<a class="bk_pop" href="#CDR0000800107_rl_676_2">2</a>-<a class="bk_pop" href="#CDR0000800107_rl_676_4">4</a>]</p><p id="CDR0000800107__1520"> One study documented the initial presentation of MEN1 syndrome occurring before age 21 years in 160 patients.[<a class="bk_pop" href="#CDR0000800107_rl_676_5">5</a>] Of note, most patients had familial MEN1 syndrome and were monitored using an international screening protocol.</p><ol id="CDR0000800107__1521"><li class="half_rhythm"><div><b> Primary hyperparathyroidism.</b> Primary hyperparathyroidism was the most common symptom. It was found in 75% of patients, usually only in those with biological abnormalities. Primary hyperparathyroidism diagnosed outside of a screening program is extremely rare, most often presents with nephrolithiasis, and should lead the clinician to suspect MEN1.[<a class="bk_pop" href="#CDR0000800107_rl_676_5">5</a>,<a class="bk_pop" href="#CDR0000800107_rl_676_6">6</a>]</div></li><li class="half_rhythm"><div>
|
|
<b>Pituitary adenomas.</b> Pituitary adenomas were discovered in 34% of patients, occurred mainly in females older than 10 years, and were often symptomatic.[<a class="bk_pop" href="#CDR0000800107_rl_676_5">5</a>]</div></li><li class="half_rhythm"><div>
|
|
<b>Pancreatic neuroendocrine tumors.</b> Pancreatic neuroendocrine tumors were found in 23% of patients. Specific diagnoses included insulinoma, nonsecreting pancreatic tumor, and Zollinger-Ellison syndrome. The first case of insulinoma occurred before age 5 years.[<a class="bk_pop" href="#CDR0000800107_rl_676_5">5</a>]</div></li><li class="half_rhythm"><div><b>Malignant tumors.</b> Four patients had malignant tumors (two adrenal carcinomas, one gastrinoma, and one thymic carcinoma). The patient with thymic carcinoma died before age 21 years of rapidly progressive disease.</div></li></ol><p id="CDR0000800107__680"><i>MEN1</i> germline mutations are found in 70% to 90% of patients; however, this gene is frequently inactivated in sporadic tumors.[<a class="bk_pop" href="#CDR0000800107_rl_676_7">7</a>] Mutation testing is combined with clinical screening for patients and family members with proven at-risk MEN1 syndrome.[<a class="bk_pop" href="#CDR0000800107_rl_676_8">8</a>] </p></div><div id="CDR0000800107__2473"><h3>MEN Type 2A (MEN2A) and MEN Type 2B (MEN2B) Syndromes</h3><p id="CDR0000800107__730">A germline activating mutation in the <i>RET</i> oncogene (a receptor tyrosine kinase) is responsible for the uncontrolled growth of cells in medullary thyroid carcinoma associated with MEN2A and MEN2B syndromes.[<a class="bk_pop" href="#CDR0000800107_rl_676_9">9</a>-<a class="bk_pop" href="#CDR0000800107_rl_676_11">11</a>] <a class="figpopup" href="/books/NBK552292.10/table/CDR0000800107__683/?report=objectonly" target="object" rid-figpopup="figCDR0000800107683" rid-ob="figobCDR0000800107683">Table 2</a> describes the clinical features of these syndromes.</p><div id="CDR0000800107__2474"><h4>MEN2A</h4><p id="CDR0000800107__2475">MEN2A is characterized by the presence of two or more endocrine tumors (see <a class="figpopup" href="/books/NBK552292.10/table/CDR0000800107__678/?report=objectonly" target="object" rid-figpopup="figCDR0000800107678" rid-ob="figobCDR0000800107678">Table 1</a>) in an individual or in close relatives.[<a class="bk_pop" href="#CDR0000800107_rl_676_12">12</a>] <i>RET</i> mutations in these patients are usually confined to exons 10 and 11. </p><ul id="CDR0000800107__2341"><li class="half_rhythm"><div class="half_rhythm"><b>Familial medullary thyroid carcinoma:</b> This carcinoma is diagnosed in families with medullary thyroid carcinoma in the absence of pheochromocytoma or parathyroid adenoma/hyperplasia. <i>RET</i> mutations in exons 10, 11, 13, and 14 account for most cases.</div><div class="half_rhythm">The most-recent literature suggests that this entity should not be identified as a form of hereditary medullary thyroid carcinoma that is separate from MEN2A and MEN2B. Familial medullary thyroid carcinoma should be recognized as a variant of MEN2A, to include families with only medullary thyroid cancer who meet the original criteria for familial disease. The original criteria includes families of at least two generations with at least two, but less
|
|
than ten, patients with <i>RET</i> germline mutations; small families in which two or fewer members in a single generation have germline <i>RET</i> mutations; and single individuals with a <i>RET</i> germline mutation.[<a class="bk_pop" href="#CDR0000800107_rl_676_13">13</a>,<a class="bk_pop" href="#CDR0000800107_rl_676_14">14</a>] </div></li></ul></div><div id="CDR0000800107__2476"><h4>MEN2B</h4><p id="CDR0000800107__2477">MEN2B is characterized by medullary thyroid carcinomas, parathyroid hyperplasias, adenomas, pheochromocytomas, mucosal neuromas, and ganglioneuromas.[<a class="bk_pop" href="#CDR0000800107_rl_676_12">12</a>,<a class="bk_pop" href="#CDR0000800107_rl_676_15">15</a>,<a class="bk_pop" href="#CDR0000800107_rl_676_16">16</a>] The medullary thyroid carcinomas that develop in these patients are extremely aggressive. More than 95% of mutations in these patients are confined to codon 918 in exon 16, causing receptor autophosphorylation and activation.[<a class="bk_pop" href="#CDR0000800107_rl_676_17">17</a>] Patients also have medullated corneal nerve fibers, distinctive faces with enlarged lips, and an asthenic Marfanoid habitus. </p><p id="CDR0000800107__1444">A pentagastrin stimulation test can be used to detect medullary thyroid carcinoma in these patients. However, the patient management is driven primarily by the results of genetic analysis for <i>RET</i> mutations.[<a class="bk_pop" href="#CDR0000800107_rl_676_14">14</a>,<a class="bk_pop" href="#CDR0000800107_rl_676_17">17</a>]</p><p id="CDR0000800107__2336">A review of 38 patients with genetically confirmed MEN2B at the National Institutes of Health identified eight patients who developed pheochromocytoma in the course of follow-up.[<a class="bk_pop" href="#CDR0000800107_rl_676_18">18</a>] Pheochromocytoma was diagnosed at a mean age of 15.2 years (± 4.6 years; range, 10–25 years) and at a mean period of 4 years (± 3.3 years) after MEN2B diagnosis. Only one patient was diagnosed with pheochromocytoma as the initial manifestation of MEN2B after she presented with hypertension and secondary amenorrhea. The youngest patient diagnosed with pheochromocytoma in this cohort was an asymptomatic child aged 10 years. The authors of this study believe that the current guidelines to begin screening for pheochromocytoma at age 11 years are appropriate.</p><p id="CDR0000800107__1525">A retrospective analysis identified 167 children with <i>RET</i> mutations who underwent prophylactic thyroidectomy. This group included 109 patients without a concomitant central node dissection and 58 patients with a concomitant central node dissection. Children were classified into risk groups by their specific type of <i>RET</i> mutation.[<a class="bk_pop" href="#CDR0000800107_rl_676_19">19</a>]</p><ul id="CDR0000800107__1526"><li class="half_rhythm"><div>In the highest-risk category, medullary thyroid carcinoma was found in five of six children (83%) aged 3 years or younger.</div></li><li class="half_rhythm"><div>In the high-risk category, medullary thyroid carcinoma was present in 6 of 20 children (30%) aged 3 years or younger, 16 of 36 children (44%) aged 4 to 6 years, and 11 of 16 children (69%) aged 7 to 12 years (<i>P</i> = .081).</div></li><li class="half_rhythm"><div>In the moderate-risk category, medullary thyroid carcinoma was seen in one of nine children (11%) aged 3 years or younger, 1 of 26 children (4%) aged 4 to 6 years, 3 of 26 children (12%) aged 7 to 12 years, and 7 of 16 children (44%) aged 13 to 18 years (<i>P</i> = .006).</div></li></ul><p id="CDR0000800107__2461"> For more information, see <a href="/books/n/pdqcis/CDR0000790382/#CDR0000790382__1905">Table 1</a> in Childhood Thyroid Cancer Treatment.</p><p id="CDR0000800107__689">In a small percentage of cases, Hirschsprung disease has been associated with the development of neuroendocrine tumors such as medullary thyroid carcinoma. <i>RET</i> germline inactivating mutations have been detected in up to 50% of patients with familial Hirschsprung disease and less often in the sporadic form.[<a class="bk_pop" href="#CDR0000800107_rl_676_20">20</a>-<a class="bk_pop" href="#CDR0000800107_rl_676_22">22</a>] Cosegregation of Hirschsprung disease and medullary thyroid carcinoma phenotype is infrequently reported, but these individuals usually have a mutation in <i>RET</i> exon 10. Patients with Hirschsprung disease are screened for mutations in <i>RET</i> exon 10. If such a mutation is discovered, a prophylactic thyroidectomy should be considered.[<a class="bk_pop" href="#CDR0000800107_rl_676_22">22</a>-<a class="bk_pop" href="#CDR0000800107_rl_676_24">24</a>]</p><p id="CDR0000800107__682">Guidelines for genetic testing of patients suspected of having MEN2 syndrome and the correlations between the type of mutation and the risk levels of aggressiveness of medullary thyroid cancer have been published.[<a class="bk_pop" href="#CDR0000800107_rl_676_14">14</a>,<a class="bk_pop" href="#CDR0000800107_rl_676_25">25</a>]</p><div id="CDR0000800107__683" class="table"><h3><span class="title">Table 2. Clinical Features of Multiple Endocrine Neoplasia Type 2 (MEN2) Syndromes<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK552292.10/table/CDR0000800107__683/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000800107__683_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="vertical-align:top;">MEN2 Subtype</th><th colspan="1" rowspan="1" style="vertical-align:top;">Medullary Thyroid Carcinoma</th><th colspan="1" rowspan="1" style="vertical-align:top;">Pheochromocytoma</th><th colspan="1" rowspan="1" style="vertical-align:top;">Parathyroid Disease</th></tr></thead><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">MEN2A</td><td colspan="1" rowspan="1" style="vertical-align:top;">95%</td><td colspan="1" rowspan="1" style="vertical-align:top;">50%</td><td colspan="1" rowspan="1" style="vertical-align:top;">20% to 30%</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">MEN2B</td><td colspan="1" rowspan="1" style="vertical-align:top;">100%</td><td colspan="1" rowspan="1" style="vertical-align:top;">50%</td><td colspan="1" rowspan="1" style="vertical-align:top;">Uncommon</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Sources: de Krijger,[<a class="bk_pop" href="#CDR0000800107_rl_676_26">26</a>] Waguespack et al.,[<a class="bk_pop" href="#CDR0000800107_rl_676_14">14</a>] Brauckhoff et al.,[<a class="bk_pop" href="#CDR0000800107_rl_676_16">16</a>] and Eng et al.[<a class="bk_pop" href="#CDR0000800107_rl_676_21">21</a>]</p></div></dd></dl></div></div></div></div></div><div id="CDR0000800107_rl_676"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800107_rl_676_1">Thakker RV: Multiple endocrine neoplasia--syndromes of the twentieth century. J Clin Endocrinol Metab 83 (8): 2617-20, 1998. [<a href="https://pubmed.ncbi.nlm.nih.gov/9709920" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9709920</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_2">Thakker RV: Multiple endocrine neoplasia type 1 (MEN1). Best Pract Res Clin Endocrinol Metab 24 (3): 355-70, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20833329" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20833329</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_3">Vannucci L, Marini F, Giusti F, et al.: MEN1 in children and adolescents: Data from patients of a regional referral center for hereditary endocrine tumors. Endocrine 59 (2): 438-448, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/28530019" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28530019</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_4">Thakker RV, Newey PJ, Walls GV, et al.: Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab 97 (9): 2990-3011, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22723327" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22723327</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_5">Goudet P, Dalac A, Le Bras M, et al.: MEN1 disease occurring before 21 years old: a 160-patient cohort study from the Groupe d'étude des Tumeurs Endocrines. J Clin Endocrinol Metab 100 (4): 1568-77, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/25594862" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25594862</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_6">Romero Arenas MA, Morris LF, Rich TA, et al.: Preoperative multiple endocrine neoplasia type 1 diagnosis improves the surgical outcomes of pediatric patients with primary hyperparathyroidism. J Pediatr Surg 49 (4): 546-50, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/24726110" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24726110</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_7">Farnebo F, Teh BT, Kytölä S, et al.: Alterations of the MEN1 gene in sporadic parathyroid tumors. J Clin Endocrinol Metab 83 (8): 2627-30, 1998. [<a href="https://pubmed.ncbi.nlm.nih.gov/9709922" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9709922</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_8">Field M, Shanley S, Kirk J: Inherited cancer susceptibility syndromes in paediatric practice. J Paediatr Child Health 43 (4): 219-29, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17444822" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17444822</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_9">Sanso GE, Domene HM, Garcia R, et al.: Very early detection of RET proto-oncogene mutation is crucial for preventive thyroidectomy in multiple endocrine neoplasia type 2 children: presence of C-cell malignant disease in asymptomatic carriers. Cancer 94 (2): 323-30, 2002. [<a href="https://pubmed.ncbi.nlm.nih.gov/11900218" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11900218</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_10">Alsanea O, Clark OH: Familial thyroid cancer. Curr Opin Oncol 13 (1): 44-51, 2001. [<a href="https://pubmed.ncbi.nlm.nih.gov/11148685" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11148685</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_11">Fitze G: Management of patients with hereditary medullary thyroid carcinoma. Eur J Pediatr Surg 14 (6): 375-83, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15630638" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15630638</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_12">Puñales MK, da Rocha AP, Meotti C, et al.: Clinical and oncological features of children and young adults with multiple endocrine neoplasia type 2A. Thyroid 18 (12): 1261-8, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18991485" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18991485</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_13">Wells SA, Asa SL, Dralle H, et al.: Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. Thyroid 25 (6): 567-610, 2015. [<a href="/pmc/articles/PMC4490627/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4490627</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/25810047" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25810047</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_14">Waguespack SG, Rich TA, Perrier ND, et al.: Management of medullary thyroid carcinoma and MEN2 syndromes in childhood. Nat Rev Endocrinol 7 (10): 596-607, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21862994" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21862994</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_15">Skinner MA, DeBenedetti MK, Moley JF, et al.: Medullary thyroid carcinoma in children with multiple endocrine neoplasia types 2A and 2B. J Pediatr Surg 31 (1): 177-81; discussion 181-2, 1996. [<a href="https://pubmed.ncbi.nlm.nih.gov/8632274" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 8632274</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_16">Brauckhoff M, Gimm O, Weiss CL, et al.: Multiple endocrine neoplasia 2B syndrome due to codon 918 mutation: clinical manifestation and course in early and late onset disease. World J Surg 28 (12): 1305-11, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15517484" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15517484</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_17">Sakorafas GH, Friess H, Peros G: The genetic basis of hereditary medullary thyroid cancer: clinical implications for the surgeon, with a particular emphasis on the role of prophylactic thyroidectomy. Endocr Relat Cancer 15 (4): 871-84, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/19015274" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19015274</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_18">Makri A, Akshintala S, Derse-Anthony C, et al.: Pheochromocytoma in Children and Adolescents With Multiple Endocrine Neoplasia Type 2B. J Clin Endocrinol Metab 104 (1): 7-12, 2019. [<a href="/pmc/articles/PMC6240163/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6240163</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/30113649" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30113649</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_19">Machens A, Elwerr M, Lorenz K, et al.: Long-term outcome of prophylactic thyroidectomy in children carrying RET germline mutations. Br J Surg 105 (2): e150-e157, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/29341155" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29341155</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_20">Decker RA, Peacock ML, Watson P: Hirschsprung disease in MEN 2A: increased spectrum of RET exon 10 genotypes and strong genotype-phenotype correlation. Hum Mol Genet 7 (1): 129-34, 1998. [<a href="https://pubmed.ncbi.nlm.nih.gov/9384613" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9384613</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_21">Eng C, Clayton D, Schuffenecker I, et al.: The relationship between specific RET proto-oncogene mutations and disease phenotype in multiple endocrine neoplasia type 2. International RET mutation consortium analysis. JAMA 276 (19): 1575-9, 1996. [<a href="https://pubmed.ncbi.nlm.nih.gov/8918855" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 8918855</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_22">Fialkowski EA, DeBenedetti MK, Moley JF, et al.: RET proto-oncogene testing in infants presenting with Hirschsprung disease identifies 2 new multiple endocrine neoplasia 2A kindreds. J Pediatr Surg 43 (1): 188-90, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18206480" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18206480</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_23">Skába R, Dvoráková S, Václavíková E, et al.: The risk of medullary thyroid carcinoma in patients with Hirschsprung's disease. Pediatr Surg Int 22 (12): 991-5, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/17021738" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17021738</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_24">Moore SW, Zaahl MG: Multiple endocrine neoplasia syndromes, children, Hirschsprung's disease and RET. Pediatr Surg Int 24 (5): 521-30, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18365214" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18365214</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_25">Kloos RT, Eng C, Evans DB, et al.: Medullary thyroid cancer: management guidelines of the American Thyroid Association. Thyroid 19 (6): 565-612, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19469690" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19469690</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_676_26">de Krijger RR: Endocrine tumor syndromes in infancy and childhood. Endocr Pathol 15 (3): 223-6, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15640547" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15640547</span></a>]</div></li></ol></div></div><div id="CDR0000800107__1864"><h2 id="_CDR0000800107__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000800107__1865">Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[<a class="bk_pop" href="#CDR0000800107_rl_1864_1">1</a>] Referral to medical centers with multidisciplinary teams of cancer specialists experienced in treating cancers that occur in childhood and adolescence should be considered. This multidisciplinary team approach incorporates the skills
|
|
of the following health care professionals and others to ensure that children receive treatment, supportive care, and rehabilitation
|
|
that will achieve optimal survival and quality of life:</p><ul id="CDR0000800107__1866"><li class="half_rhythm"><div>Primary care physicians.</div></li><li class="half_rhythm"><div>Pediatric surgeons.</div></li><li class="half_rhythm"><div>Radiation
|
|
oncologists.</div></li><li class="half_rhythm"><div>Pediatric medical oncologists/hematologists.</div></li><li class="half_rhythm"><div> Rehabilitation
|
|
specialists.</div></li><li class="half_rhythm"><div>Pediatric nurse specialists.</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></ul><p id="CDR0000800107__1867">For 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="CDR0000800107__1868">The American Academy of Pediatrics has outlined guidelines for
|
|
pediatric cancer centers and their role in the treatment of pediatric patients
|
|
with cancer.[<a class="bk_pop" href="#CDR0000800107_rl_1864_2">2</a>] At
|
|
these pediatric cancer 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. Most of the progress made in identifying curative
|
|
therapy for childhood cancers has been achieved through clinical trials.
|
|
Information about ongoing clinical trials is available from the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>.</p><p id="CDR0000800107__1869">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="#CDR0000800107_rl_1864_3">3</a>-<a class="bk_pop" href="#CDR0000800107_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="CDR0000800107__1870">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="#CDR0000800107_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. Therefore, all pediatric cancers are considered rare.</p><p id="CDR0000800107__1888">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="#CDR0000800107_rl_1864_7">7</a>,<a class="bk_pop" href="#CDR0000800107_rl_1864_8">8</a>] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000800107__1871"><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 2 cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than 2 cases per 1 million people were also included in the very rare group because there is a lack of knowledge and expertise in the management of these tumors.[<a class="bk_pop" href="#CDR0000800107_rl_1864_9">9</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">The Children's Oncology Group (COG) 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, colorectal cancers, etc.).[<a class="bk_pop" href="#CDR0000800107_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="#CDR0000800107_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 in children aged 0 to 14 years and 9.3% of the cancers in adolescents aged 15 to 19 years.</div></li></ul><p id="CDR0000800107__1872">These rare cancers are extremely challenging to study because of the low number of patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the lack of clinical trials for adolescents with rare cancers.</p><div id="CDR0000800107_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800107_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="CDR0000800107_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 December 15, 2023.</div></li><li><div class="bk_ref" id="CDR0000800107_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="CDR0000800107_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 December 15, 2023.</div></li><li><div class="bk_ref" id="CDR0000800107_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 August 18, 2023.</div></li><li><div class="bk_ref" id="CDR0000800107_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="CDR0000800107_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="CDR0000800107_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="CDR0000800107_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="CDR0000800107_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="CDR0000800107__685"><h2 id="_CDR0000800107__685_">Treatment of Childhood Multiple Endocrine Neoplasia (MEN) Syndromes</h2><p id="CDR0000800107__2325">Treatment options for childhood MEN syndromes, according to type, are as follows:</p><div id="CDR0000800107__2462"><h3>MEN Type 1 (MEN1) Syndrome</h3><p id="CDR0000800107__2463"> The treatment of patients with MEN1 syndrome is based on the type of tumor. The outcomes
|
|
of patients with MEN1 syndrome are generally good provided adequate
|
|
treatment can be obtained for parathyroid, pancreatic, and pituitary tumors.</p><p id="CDR0000800107__1301">The standard approach to patients who present with hyperparathyroidism and MEN1 syndrome is genetic testing and treatment with a cervical resection of at least three parathyroid glands and transcervical thymectomy.[<a class="bk_pop" href="#CDR0000800107_rl_685_1">1</a>]</p><p id="CDR0000800107__2464"> For more information, see the <a href="/books/n/pdqcis/CDR0000062890/#CDR0000062890__777">Interventions</a> section in Genetics of Endocrine and Neuroendocrine Neoplasias.</p></div><div id="CDR0000800107__2465"><h3>MEN Type 2 (MEN2) Syndromes</h3><p id="CDR0000800107__2466">The management of medullary thyroid cancer in children from families having MEN2 syndromes relies on presymptomatic detection of the <i>RET</i> proto-oncogene mutation responsible for the disease. </p><div id="CDR0000800107__2467"><h4>MEN2A syndrome</h4><p id="CDR0000800107__2468">For children with MEN2A, thyroidectomy is commonly performed by approximately age 5 years or older if that is when a <i>RET</i> mutation is identified.[<a class="bk_pop" href="#CDR0000800107_rl_685_2">2</a>-<a class="bk_pop" href="#CDR0000800107_rl_685_7">7</a>] The outcomes for patients with MEN2A syndrome are generally good, although recurrences of medullary thyroid carcinoma and
|
|
pheochromocytoma can occur.[<a class="bk_pop" href="#CDR0000800107_rl_685_8">8</a>-<a class="bk_pop" href="#CDR0000800107_rl_685_10">10</a>]</p><p id="CDR0000800107__844">A retrospective analysis identified 262 patients with MEN2A syndrome.[<a class="bk_pop" href="#CDR0000800107_rl_685_11">11</a>] The median age of the cohort was 42 years and ranged from age 6 to 86 years. There was no correlation between the specific <i>RET</i> mutation identified and the risk of distant metastasis. Younger age at diagnosis did increase the risk of distant metastasis. </p><p id="CDR0000800107__2487">Young children who are relatives of patients with MEN2A undergo genetic testing before the age of 5 years. Carriers undergo total thyroidectomy as described above, with autotransplantation of one parathyroid gland by a certain age.[<a class="bk_pop" href="#CDR0000800107_rl_685_12">12</a>-<a class="bk_pop" href="#CDR0000800107_rl_685_15">15</a>]</p></div><div id="CDR0000800107__2469"><h4>MEN2B syndrome</h4><p id="CDR0000800107__2470"> Patients with MEN2B syndrome have worse outcomes primarily because medullary thyroid carcinoma is more aggressive. Because of the increased severity of medullary thyroid carcinoma in children with MEN2B and in those with <i>RET</i> mutations in codons 883, 918, and 922, it is recommended that these children undergo prophylactic thyroidectomy in infancy.[<a class="bk_pop" href="#CDR0000800107_rl_685_3">3</a>,<a class="bk_pop" href="#CDR0000800107_rl_685_16">16</a>,<a class="bk_pop" href="#CDR0000800107_rl_685_17">17</a>]; [<a class="bk_pop" href="#CDR0000800107_rl_685_18">18</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810037/" class="def">Level of evidence C2</a>] This can improve outcomes in patients with MEN2B.[<a class="bk_pop" href="#CDR0000800107_rl_685_19">19</a>] Complete removal of the thyroid gland is the recommended surgical management of medullary thyroid cancer in children because there is a high incidence of bilateral disease.</p></div><div id="CDR0000800107__2478"><h4>Targeted therapy</h4><p id="CDR0000800107__2479">Targeted therapy has been used for patients with the <i>RET</i> gene mutation and medullary thyroid cancer. Types of targeted therapy include the following:</p><div id="CDR0000800107__2480"><h5>Vandetanib (a kinase inhibitor)</h5><p id="CDR0000800107__2481">A randomized phase III trial included adult patients with unresectable locally advanced or metastatic hereditary or sporadic medullary thyroid carcinoma who were treated with either vandetanib (a selective inhibitor of RET, vascular endothelial growth factor receptor, and epidermal growth factor receptor) or placebo. The study found that vandetanib administration was associated with significant improvements in progression-free survival (PFS), response rate, disease control rates, and biochemical response.[<a class="bk_pop" href="#CDR0000800107_rl_685_20">20</a>] </p><p id="CDR0000800107__2482">Children with locally advanced or metastatic medullary thyroid carcinoma were treated with vandetanib in a phase I/II trial.[<a class="bk_pop" href="#CDR0000800107_rl_685_21">21</a>]</p><ul id="CDR0000800107__2483"><li class="half_rhythm"><div> Of 16 patients, only the patient without the M918T <i>RET</i> mutation had no response.</div></li><li class="half_rhythm"><div>Of the 15 patients who had a tumor response, seven had a partial response.</div></li><li class="half_rhythm"><div> Three of the 15 patients had subsequent disease recurrence.</div></li><li class="half_rhythm"><div> Eleven of the 16 patients treated with vandetanib remained on therapy at the time of the report.</div></li><li class="half_rhythm"><div> A subsequent follow-up analysis of this cohort plus one additional patient revealed that 10 of the 17 patients achieved a partial response, and an additional 6 individuals had stable disease. The median PFS for these patients was 6.7 years.[<a class="bk_pop" href="#CDR0000800107_rl_685_22">22</a>]</div></li></ul></div><div id="CDR0000800107__2484"><h5>Selpercatinib (a RET inhibitor)</h5><p id="CDR0000800107__2485">A phase I/II trial of selpercatinib therapy included patients with <i>RET</i>-mutant cancers. The study enrolled 55 patients with medullary thyroid cancer (age range, 17–84 years) who were previously treated with vandetanib and/or cabozantinib and 88 patients with medullary thyroid cancer (age range, 15–82 years) who were not previously treated with vandetanib or cabozantinib.[<a class="bk_pop" href="#CDR0000800107_rl_685_23">23</a>]</p><ul id="CDR0000800107__2344"><li class="half_rhythm"><div>For the previously treated cohort, 69% of patients achieved an objective response, and the median duration of response had not been reached, with a median follow-up of 14 months. </div></li><li class="half_rhythm"><div>For the cohort of patients who were not previously treated, 73% of patients achieved an objective response, with a median duration of response of 22.0 months.</div></li><li class="half_rhythm"><div>The most common grades 3 to 4 treatment-related adverse events were hypertension (12%), increased alanine aminotransferase (10%) and aspartate aminotransferase (7%), diarrhea (3%), and prolonged QT interval (2%). </div></li><li class="half_rhythm"><div>The U.S. Food and Drug Administration granted accelerated approval of selpercatinib for the treatment of adult and pediatric patients aged 12 years and older with advanced or metastatic <i>RET</i>-mutant medullary thyroid cancer who require systemic therapy.[<a class="bk_pop" href="#CDR0000800107_rl_685_24">24</a>]</div></li></ul></div></div></div><div id="CDR0000800107_rl_685"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800107_rl_685_1">Romero Arenas MA, Morris LF, Rich TA, et al.: Preoperative multiple endocrine neoplasia type 1 diagnosis improves the surgical outcomes of pediatric patients with primary hyperparathyroidism. J Pediatr Surg 49 (4): 546-50, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/24726110" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24726110</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_2">Skinner MA, Moley JA, Dilley WG, et al.: Prophylactic thyroidectomy in multiple endocrine neoplasia type 2A. N Engl J Med 353 (11): 1105-13, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/16162881" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16162881</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_3">Skinner MA: Management of hereditary thyroid cancer in children. Surg Oncol 12 (2): 101-4, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12946481" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12946481</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_4">Fitze G: Management of patients with hereditary medullary thyroid carcinoma. Eur J Pediatr Surg 14 (6): 375-83, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15630638" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15630638</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_5">Learoyd DL, Gosnell J, Elston MS, et al.: Experience of prophylactic thyroidectomy in multiple endocrine neoplasia type 2A kindreds with RET codon 804 mutations. Clin Endocrinol (Oxf) 63 (6): 636-41, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/16343097" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16343097</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_6">Guillem JG, Wood WC, Moley JF, et al.: ASCO/SSO review of current role of risk-reducing surgery in common hereditary cancer syndromes. J Clin Oncol 24 (28): 4642-60, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/17008706" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17008706</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_7">National Comprehensive Cancer Network: NCCN Clinical Practice Guidelines in Oncology: Thyroid Carcinoma. Version 2.2019. Plymouth Meeting, Pa: National Comprehensive Cancer Network, 2019. <a href="https://www.nccn.org/professionals/physician_gls/pdf/thyroid.pdf" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online with free subscription.</a> Last accessed June 08, 2020. [<a href="https://pubmed.ncbi.nlm.nih.gov/35948029" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 35948029</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_8">Lallier M, St-Vil D, Giroux M, et al.: Prophylactic thyroidectomy for medullary thyroid carcinoma in gene carriers of MEN2 syndrome. J Pediatr Surg 33 (6): 846-8, 1998. [<a href="https://pubmed.ncbi.nlm.nih.gov/9660211" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9660211</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_9">Dralle H, Gimm O, Simon D, et al.: Prophylactic thyroidectomy in 75 children and adolescents with hereditary medullary thyroid carcinoma: German and Austrian experience. World J Surg 22 (7): 744-50; discussion 750-1, 1998. [<a href="https://pubmed.ncbi.nlm.nih.gov/9606292" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9606292</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_10">Skinner MA, Wells SA: Medullary carcinoma of the thyroid gland and the MEN 2 syndromes. Semin Pediatr Surg 6 (3): 134-40, 1997. [<a href="https://pubmed.ncbi.nlm.nih.gov/9263335" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9263335</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_11">Voss RK, Feng L, Lee JE, et al.: Medullary Thyroid Carcinoma in MEN2A: ATA Moderate- or High-Risk RET Mutations Do Not Predict Disease Aggressiveness. J Clin Endocrinol Metab 102 (8): 2807-2813, 2017. [<a href="/pmc/articles/PMC5546858/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5546858</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/28609830" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28609830</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_12">Heizmann O, Haecker FM, Zumsteg U, et al.: Presymptomatic thyroidectomy in multiple endocrine neoplasia 2a. Eur J Surg Oncol 32 (1): 98-102, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16325365" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16325365</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_13">Frank-Raue K, Buhr H, Dralle H, et al.: Long-term outcome in 46 gene carriers of hereditary medullary thyroid carcinoma after prophylactic thyroidectomy: impact of individual RET genotype. Eur J Endocrinol 155 (2): 229-36, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16868135" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16868135</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_14">Piolat C, Dyon JF, Sturm N, et al.: Very early prophylactic thyroid surgery for infants with a mutation of the RET proto-oncogene at codon 634: evaluation of the implementation of international guidelines for MEN type 2 in a single centre. Clin Endocrinol (Oxf) 65 (1): 118-24, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16817830" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16817830</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_15">National Comprehensive Cancer Network: NCCN Clinical Practice Guidelines in Oncology: Thyroid Carcinoma. Version 1.2018. Fort Washington, Pa: National Comprehensive Cancer Network, 2018. <a href="https://www.nccn.org/professionals/physician_gls/pdf/thyroid.pdf" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online with free subscription.</a> Last accessed July 5, 2018.</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_16">Leboulleux S, Travagli JP, Caillou B, et al.: Medullary thyroid carcinoma as part of a multiple endocrine neoplasia type 2B syndrome: influence of the stage on the clinical course. Cancer 94 (1): 44-50, 2002. [<a href="https://pubmed.ncbi.nlm.nih.gov/11815959" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11815959</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_17">Sakorafas GH, Friess H, Peros G: The genetic basis of hereditary medullary thyroid cancer: clinical implications for the surgeon, with a particular emphasis on the role of prophylactic thyroidectomy. Endocr Relat Cancer 15 (4): 871-84, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/19015274" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19015274</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_18">Zenaty D, Aigrain Y, Peuchmaur M, et al.: Medullary thyroid carcinoma identified within the first year of life in children with hereditary multiple endocrine neoplasia type 2A (codon 634) and 2B. Eur J Endocrinol 160 (5): 807-13, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19240193" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19240193</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_19">Brauckhoff M, Machens A, Lorenz K, et al.: Surgical curability of medullary thyroid cancer in multiple endocrine neoplasia 2B: a changing perspective. Ann Surg 259 (4): 800-6, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/23979292" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23979292</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_20">Wells SA, Robinson BG, Gagel RF, et al.: Vandetanib in patients with locally advanced or metastatic medullary thyroid cancer: a randomized, double-blind phase III trial. J Clin Oncol 30 (2): 134-41, 2012. [<a href="/pmc/articles/PMC3675689/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3675689</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/22025146" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22025146</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_21">Fox E, Widemann BC, Chuk MK, et al.: Vandetanib in children and adolescents with multiple endocrine neoplasia type 2B associated medullary thyroid carcinoma. Clin Cancer Res 19 (15): 4239-48, 2013. [<a href="/pmc/articles/PMC4274128/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4274128</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/23766359" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23766359</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_22">Kraft IL, Akshintala S, Zhu Y, et al.: Outcomes of Children and Adolescents with Advanced Hereditary Medullary Thyroid Carcinoma Treated with Vandetanib. Clin Cancer Res 24 (4): 753-765, 2018. [<a href="/pmc/articles/PMC5815946/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5815946</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/29187393" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29187393</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_23">Wirth LJ, Sherman E, Robinson B, et al.: Efficacy of Selpercatinib in RET-Altered Thyroid Cancers. N Engl J Med 383 (9): 825-835, 2020. [<a href="https://pubmed.ncbi.nlm.nih.gov/32846061" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 32846061</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800107_rl_685_24">Eli Lilly and Company: RETEVMO (selpercatinib): Prescribing Information. Indianapolis, Ind: Lilly USA, LLC, 2020. <a href="https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/213246s000lbl.pdf" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online</a>. Last accessed September 28, 2020.</div></li></ol></div></div><div id="CDR0000800107__1985"><h2 id="_CDR0000800107__1985_">Treatment Options Under Clinical Evaluation for Multiple Endocrine Neoplasia (MEN) Syndromes</h2><p id="CDR0000800107__1986">Information about National Cancer Institute (NCI)–supported clinical trials can be found on the <a href="https://www.cancer.gov/about-cancer/treatment/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="CDR0000800107__1987">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000800107__1988"><li class="half_rhythm"><div><b><a href="https://www.cancer.gov/clinicaltrials/NCT03157128" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">LIBRETTO-001 (NCT03157128)</a></b> (Phase I/II Study of LOXO-292 in Patients With Advanced Solid Tumors, <i>RET</i> Fusion–Positive Solid Tumors, and Medullary Thyroid Cancer)<b>:</b> This is a phase I/II, open-label, first-in-human study designed to evaluate the safety, tolerability, pharmacokinetics, and preliminary antitumor activity of selpercatinib (also known as LOXO-292) administered orally to patients with advanced solid tumors, including <i>RET</i> fusion–positive solid tumors, medullary thyroid cancer, and other tumors with <i>RET</i> activation.</div></li></ul></div><div id="CDR0000800107__2331"><h2 id="_CDR0000800107__2331_">Latest Updates to This Summary (01/03/2024)</h2><p id="CDR0000800107__2332">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="CDR0000800107__2488">Editorial changes were made to this summary.</p><p id="CDR0000800107__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="#CDR0000800107__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="CDR0000800107__AboutThis_1"><h2 id="_CDR0000800107__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000800107__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000800107__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric multiple endocrine neoplasia (MEN) syndromes. 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="CDR0000800107__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000800107__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="CDR0000800107__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000800107__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="CDR0000800107__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 Multiple Endocrine Neoplasia (MEN) Syndromes 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 Cancer Institute/Boston Children's Hospital)</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="CDR0000800107__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="CDR0000800107__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000800107__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="CDR0000800107__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000800107__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="CDR0000800107__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000800107__AboutThis_15">PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/multiple-endocrine-neoplasia/hp-child-men-syndromes-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/multiple-endocrine-neoplasia/hp-child-men-syndromes-treatment-pdq</a>. Accessed <MM/DD/YYYY>. [PMID: 31909948]</p><p id="CDR0000800107__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="CDR0000800107__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000800107__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="CDR0000800107__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000800107__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: NBK552292</span><span class="label">PMID: <a href="https://pubmed.ncbi.nlm.nih.gov/31909948" title="PubMed record of this page" ref="pagearea=meta&targetsite=entrez&targetcat=link&targettype=pubmed">31909948</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=NBK552292&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/NBK552292.10/?report=reader">PubReader</a></li><li><a href="/books/NBK552292.10/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK552292" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK552292" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Pediatric Treatment Editorial Board. Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment (PDQ®): Health Professional Version. 2024 Jan 3. 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/NBK552292.15/">NBK552292.15</a></span> December 23, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK552292.14/">NBK552292.14</a></span> October 17, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK552292.13/">NBK552292.13</a></span> September 24, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK552292.12/">NBK552292.12</a></span> April 26, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK552292.11/">NBK552292.11</a></span> January 31, 2024</li><li><span class="bk_col_itm">NBK552292.10</span> January 3, 2024 (Displayed Version)</li><li><span class="bk_col_itm"><a href="/books/NBK552292.9/">NBK552292.9</a></span> January 6, 2023</li><li><span class="bk_col_itm"><a href="/books/NBK552292.8/">NBK552292.8</a></span> December 13, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK552292.7/">NBK552292.7</a></span> February 25, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK552292.6/">NBK552292.6</a></span> December 17, 2021</li><li><span class="bk_col_itm"><a href="/books/NBK552292.5/">NBK552292.5</a></span> October 6, 2021</li><li><span class="bk_col_itm"><a href="/books/NBK552292.4/">NBK552292.4</a></span> February 11, 2021</li><li><span class="bk_col_itm"><a href="/books/NBK552292.3/">NBK552292.3</a></span> October 6, 2020</li><li><span class="bk_col_itm"><a href="/books/NBK552292.2/">NBK552292.2</a></span> June 8, 2020</li><li><span class="bk_col_itm"><a href="/books/NBK552292.1/">NBK552292.1</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="#CDR0000800107__2333" ref="log$=inpage&link_id=inpage">General Information About Childhood Multiple Endocrine Neoplasia (MEN) Syndromes</a></li><li><a href="#CDR0000800107__676" ref="log$=inpage&link_id=inpage">Clinical Presentation, Molecular Features, and Diagnostic Evaluation</a></li><li><a href="#CDR0000800107__1864" ref="log$=inpage&link_id=inpage">Special Considerations for the Treatment of Children With Cancer</a></li><li><a href="#CDR0000800107__685" ref="log$=inpage&link_id=inpage">Treatment of Childhood Multiple Endocrine Neoplasia (MEN) Syndromes</a></li><li><a href="#CDR0000800107__1985" ref="log$=inpage&link_id=inpage">Treatment Options Under Clinical Evaluation for Multiple Endocrine Neoplasia (MEN) Syndromes</a></li><li><a href="#CDR0000800107__2331" ref="log$=inpage&link_id=inpage">Latest Updates to This Summary (01/03/2024)</a></li><li><a href="#CDR0000800107__AboutThis_1" ref="log$=inpage&link_id=inpage">About This PDQ Summary</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related publications</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="document-links" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="/books/NBK552281/">Patient Version</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related information</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="discovery_db_links" id="Shutter"></a></div><div class="portlet_content"><ul><li class="brieflinkpopper"><a class="brieflinkpopperctrl" href="/books/?Db=pmc&DbFrom=books&Cmd=Link&LinkName=books_pmc_refs&IdsFromResult=4911122" ref="log$=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=4911122" ref="log$=recordlinks">PubMed</a><div class="brieflinkpop offscreen_noflow">Links to PubMed</div></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Similar articles in PubMed</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="PBooksDiscovery_RA" id="Shutter"></a></div><div class="portlet_content"><ul><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/39689224" ref="ordinalpos=1&linkpos=1&log$=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> Multiple Endocrine Neoplasia Type 2 (MEN2) (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> Multiple Endocrine Neoplasia Type 2 (MEN2) (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 Cancer Genetics 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/38630971" ref="ordinalpos=1&linkpos=2&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Myelodysplastic Neoplasms Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Myelodysplastic Neoplasms Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389243" ref="ordinalpos=1&linkpos=3&log$=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 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/31909942" ref="ordinalpos=1&linkpos=4&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Pheochromocytoma and Paraganglioma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Pheochromocytoma and Paraganglioma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/31593397" ref="ordinalpos=1&linkpos=5&log$=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 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></ul><a class="seemore" href="/sites/entrez?db=pubmed&cmd=link&linkname=pubmed_pubmed_reviews&uid=31909948" ref="ordinalpos=1&log$=relatedreviews_seeall&logdbfrom=pubmed">See reviews...</a><a class="seemore" href="/sites/entrez?db=pubmed&cmd=link&linkname=pubmed_pubmed&uid=31909948" ref="ordinalpos=1&log$=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$=activity&linkpos=1" href="/portal/utils/pageresolver.fcgi?recordid=67c9958684f3725e5920ada5">Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment (PDQ®) - PDQ Ca...</a><div class="ralinkpop offscreen_noflow">Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment (PDQ®) - PDQ Cancer Information Summaries<div class="brieflinkpopdesc"></div></div><div class="tertiary"></div></li><li class="ra_qry two_line"><a class="htb" ref="log$=activity&linkpos=2" href="/portal/utils/pageresolver.fcgi?recordid=67c9956484f3725e591fb84f">PMC Links for Books (Select 2824476) <span class="number">(1)</span></a><div class="tertiary">PMC</div></li><li class="ra_rcd ralinkpopper two_line"><a class="htb ralinkpopperctrl" ref="log$=activity&linkpos=3" href="/portal/utils/pageresolver.fcgi?recordid=67c99562f4a390645e1ca628">Metastatic Squamous Neck Cancer With Occult Primary Treatment (PDQ®) - PDQ Cance...</a><div class="ralinkpop offscreen_noflow">Metastatic Squamous Neck Cancer With Occult Primary Treatment (PDQ®) - PDQ Cancer Information Summaries<div class="brieflinkpopdesc"></div></div><div class="tertiary"></div></li><li class="ra_rcd ralinkpopper two_line"><a class="htb ralinkpopperctrl" ref="log$=activity&linkpos=4" href="/portal/utils/pageresolver.fcgi?recordid=67c99560b15b832ebcf1192e">Metastatic Squamous Neck Cancer With Occult Primary Treatment (Adult) (PDQ®) - P...</a><div class="ralinkpop offscreen_noflow">Metastatic Squamous Neck Cancer With Occult Primary Treatment (Adult) (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$=activity&linkpos=5" href="/portal/utils/pageresolver.fcgi?recordid=67c9952484f3725e591e0114">PMC Links for Books (Select 2822696) <span class="number">(3)</span></a><div class="tertiary">PMC</div></li></ul><p class="HTOn">Your browsing activity is empty.</p><p class="HTOff">Activity recording is turned off.</p><p id="turnOn" class="HTOff"><a href="javascript:historyDisplayState('HTOn')">Turn recording back on</a></p><a class="seemore" href="/sites/myncbi/recentactivity">See more...</a></div></div></div>
|
|
|
|
<!-- Custom content below discovery portlets -->
|
|
<div class="col7">
|
|
|
|
</div>
|
|
</div>
|
|
</div>
|
|
|
|
<!-- Custom content after all -->
|
|
<div class="col8">
|
|
|
|
</div>
|
|
<div class="col9">
|
|
|
|
</div>
|
|
|
|
<script type="text/javascript" src="/corehtml/pmc/js/jquery.scrollTo-1.4.2.js"></script>
|
|
<script type="text/javascript">
|
|
(function($){
|
|
$('.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=NBK552292&ncbi_domain=pdqcis&ncbi_report=record&ncbi_type=fulltext&ncbi_objectid=&ncbi_pcid=/NBK552292.10/&ncbi_pagename=Childhood Multiple Endocrine Neoplasia (MEN) Syndromes 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 portal106 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> |