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id="toc-Embryonal" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Embryonal"> <div class="vector-toc-text"> <span class="vector-toc-numb">1.1</span> <span>Embryonal</span> </div> </a> <ul id="toc-Embryonal-sublist" class="vector-toc-list"> <li id="toc-Embryonal_subtype" class="vector-toc-list-item vector-toc-level-3"> <a class="vector-toc-link" href="#Embryonal_subtype"> <div class="vector-toc-text"> <span class="vector-toc-numb">1.1.1</span> <span>Embryonal subtype</span> </div> </a> <ul id="toc-Embryonal_subtype-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Alveolar" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Alveolar"> <div class="vector-toc-text"> <span class="vector-toc-numb">1.2</span> <span>Alveolar</span> </div> </a> <ul id="toc-Alveolar-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Pleomorphic" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Pleomorphic"> <div class="vector-toc-text"> <span class="vector-toc-numb">1.3</span> <span>Pleomorphic</span> </div> </a> <ul id="toc-Pleomorphic-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Spindle-cell/sclerosing" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Spindle-cell/sclerosing"> <div class="vector-toc-text"> <span class="vector-toc-numb">1.4</span> <span>Spindle-cell/sclerosing</span> </div> </a> <ul id="toc-Spindle-cell/sclerosing-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Signs_and_symptoms" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Signs_and_symptoms"> <div class="vector-toc-text"> <span class="vector-toc-numb">2</span> <span>Signs and symptoms</span> </div> </a> <ul id="toc-Signs_and_symptoms-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Risk_factors" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Risk_factors"> <div class="vector-toc-text"> <span class="vector-toc-numb">3</span> <span>Risk factors</span> </div> </a> <ul id="toc-Risk_factors-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Genetic" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Genetic"> <div class="vector-toc-text"> <span class="vector-toc-numb">4</span> <span>Genetic</span> </div> </a> <ul id="toc-Genetic-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Diagnosis" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Diagnosis"> <div class="vector-toc-text"> <span class="vector-toc-numb">5</span> <span>Diagnosis</span> </div> </a> <button aria-controls="toc-Diagnosis-sublist" class="cdx-button cdx-button--weight-quiet cdx-button--icon-only vector-toc-toggle"> <span class="vector-icon mw-ui-icon-wikimedia-expand"></span> <span>Toggle Diagnosis subsection</span> </button> <ul id="toc-Diagnosis-sublist" class="vector-toc-list"> <li id="toc-Staging" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Staging"> <div class="vector-toc-text"> <span class="vector-toc-numb">5.1</span> <span>Staging</span> </div> </a> <ul id="toc-Staging-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Treatment" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Treatment"> <div class="vector-toc-text"> <span class="vector-toc-numb">6</span> <span>Treatment</span> </div> </a> <ul id="toc-Treatment-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Prognostic" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Prognostic"> <div class="vector-toc-text"> <span class="vector-toc-numb">7</span> <span>Prognostic</span> </div> </a> <ul id="toc-Prognostic-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Epidemiology" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Epidemiology"> <div class="vector-toc-text"> <span class="vector-toc-numb">8</span> <span>Epidemiology</span> </div> </a> <ul id="toc-Epidemiology-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-History" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#History"> <div class="vector-toc-text"> <span class="vector-toc-numb">9</span> <span>History</span> </div> </a> <ul id="toc-History-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Research" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Research"> <div class="vector-toc-text"> <span class="vector-toc-numb">10</span> <span>Research</span> </div> </a> <ul id="toc-Research-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-See_also" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#See_also"> <div class="vector-toc-text"> <span class="vector-toc-numb">11</span> <span>See also</span> </div> </a> <ul id="toc-See_also-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-References" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#References"> <div class="vector-toc-text"> <span class="vector-toc-numb">12</span> <span>References</span> </div> </a> <ul id="toc-References-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-External_links" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#External_links"> <div class="vector-toc-text"> <span class="vector-toc-numb">13</span> <span>External links</span> </div> </a> <ul id="toc-External_links-sublist" class="vector-toc-list"> </ul> </li> </ul> </div> </div> </nav> </div> </div> <div class="mw-content-container"> <main id="content" class="mw-body"> <header class="mw-body-header vector-page-titlebar"> <nav aria-label="Contents" class="vector-toc-landmark"> <div id="vector-page-titlebar-toc" class="vector-dropdown vector-page-titlebar-toc vector-button-flush-left" > <input type="checkbox" id="vector-page-titlebar-toc-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-vector-page-titlebar-toc" class="vector-dropdown-checkbox " aria-label="Toggle the table of contents" > <label id="vector-page-titlebar-toc-label" for="vector-page-titlebar-toc-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--icon-only " aria-hidden="true" ><span class="vector-icon mw-ui-icon-listBullet mw-ui-icon-wikimedia-listBullet"></span> <span 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Available in 22 languages" > <label id="p-lang-btn-label" for="p-lang-btn-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--action-progressive mw-portlet-lang-heading-22" aria-hidden="true" ><span class="vector-icon mw-ui-icon-language-progressive mw-ui-icon-wikimedia-language-progressive"></span> <span class="vector-dropdown-label-text">22 languages</span> </label> <div class="vector-dropdown-content"> <div class="vector-menu-content"> <ul class="vector-menu-content-list"> <li class="interlanguage-link interwiki-ar mw-list-item"><a href="https://ar.wikipedia.org/wiki/%D8%A7%D9%84%D8%B3%D8%A7%D8%B1%D9%83%D9%88%D9%85%D8%A9_%D8%A7%D9%84%D8%B9%D8%B6%D9%84%D9%8A%D8%A9_%D8%A7%D9%84%D9%85%D8%AE%D8%B7%D8%B7%D8%A9" title="الساركومة العضلية المخططة – Arabic" lang="ar" hreflang="ar" data-title="الساركومة العضلية المخططة" data-language-autonym="العربية" data-language-local-name="Arabic" class="interlanguage-link-target"><span>العربية</span></a></li><li class="interlanguage-link interwiki-de mw-list-item"><a href="https://de.wikipedia.org/wiki/Rhabdomyosarkom" title="Rhabdomyosarkom – German" lang="de" hreflang="de" data-title="Rhabdomyosarkom" data-language-autonym="Deutsch" data-language-local-name="German" class="interlanguage-link-target"><span>Deutsch</span></a></li><li class="interlanguage-link interwiki-es mw-list-item"><a href="https://es.wikipedia.org/wiki/Rabdomiosarcoma" title="Rabdomiosarcoma – Spanish" lang="es" hreflang="es" data-title="Rabdomiosarcoma" data-language-autonym="Español" data-language-local-name="Spanish" class="interlanguage-link-target"><span>Español</span></a></li><li class="interlanguage-link interwiki-eu mw-list-item"><a href="https://eu.wikipedia.org/wiki/Errabdomiosarkoma" title="Errabdomiosarkoma – Basque" lang="eu" hreflang="eu" data-title="Errabdomiosarkoma" data-language-autonym="Euskara" data-language-local-name="Basque" class="interlanguage-link-target"><span>Euskara</span></a></li><li class="interlanguage-link interwiki-fr mw-list-item"><a href="https://fr.wikipedia.org/wiki/Rhabdomyosarcome" title="Rhabdomyosarcome – French" lang="fr" hreflang="fr" data-title="Rhabdomyosarcome" data-language-autonym="Français" data-language-local-name="French" class="interlanguage-link-target"><span>Français</span></a></li><li class="interlanguage-link interwiki-ko mw-list-item"><a href="https://ko.wikipedia.org/wiki/%ED%9A%A1%EB%AC%B8%EA%B7%BC%EC%9C%A1%EC%A2%85" title="횡문근육종 – Korean" lang="ko" hreflang="ko" data-title="횡문근육종" data-language-autonym="한국어" data-language-local-name="Korean" class="interlanguage-link-target"><span>한국어</span></a></li><li class="interlanguage-link interwiki-it mw-list-item"><a href="https://it.wikipedia.org/wiki/Rabdomiosarcoma" title="Rabdomiosarcoma – Italian" lang="it" hreflang="it" data-title="Rabdomiosarcoma" data-language-autonym="Italiano" data-language-local-name="Italian" class="interlanguage-link-target"><span>Italiano</span></a></li><li class="interlanguage-link interwiki-nl mw-list-item"><a href="https://nl.wikipedia.org/wiki/Rabdomyosarcoom" title="Rabdomyosarcoom – Dutch" lang="nl" hreflang="nl" data-title="Rabdomyosarcoom" data-language-autonym="Nederlands" data-language-local-name="Dutch" class="interlanguage-link-target"><span>Nederlands</span></a></li><li class="interlanguage-link interwiki-ja mw-list-item"><a href="https://ja.wikipedia.org/wiki/%E6%A8%AA%E7%B4%8B%E7%AD%8B%E8%82%89%E8%85%AB" title="横紋筋肉腫 – Japanese" lang="ja" hreflang="ja" data-title="横紋筋肉腫" data-language-autonym="日本語" data-language-local-name="Japanese" class="interlanguage-link-target"><span>日本語</span></a></li><li class="interlanguage-link interwiki-no mw-list-item"><a href="https://no.wikipedia.org/wiki/Rhabdomyosarkom" title="Rhabdomyosarkom – Norwegian Bokmål" lang="nb" hreflang="nb" data-title="Rhabdomyosarkom" data-language-autonym="Norsk bokmål" data-language-local-name="Norwegian Bokmål" class="interlanguage-link-target"><span>Norsk bokmål</span></a></li><li class="interlanguage-link interwiki-pl mw-list-item"><a href="https://pl.wikipedia.org/wiki/Mi%C4%99%C5%9Bniakomi%C4%99sak_pr%C4%85%C5%BCkowanokom%C3%B3rkowy" title="Mięśniakomięsak prążkowanokomórkowy – Polish" lang="pl" hreflang="pl" data-title="Mięśniakomięsak prążkowanokomórkowy" data-language-autonym="Polski" data-language-local-name="Polish" class="interlanguage-link-target"><span>Polski</span></a></li><li class="interlanguage-link interwiki-pt mw-list-item"><a href="https://pt.wikipedia.org/wiki/Rabdomiossarcoma" title="Rabdomiossarcoma – Portuguese" lang="pt" hreflang="pt" data-title="Rabdomiossarcoma" data-language-autonym="Português" data-language-local-name="Portuguese" class="interlanguage-link-target"><span>Português</span></a></li><li class="interlanguage-link interwiki-ro mw-list-item"><a href="https://ro.wikipedia.org/wiki/Rabdomiosarcom" title="Rabdomiosarcom – Romanian" lang="ro" hreflang="ro" data-title="Rabdomiosarcom" data-language-autonym="Română" data-language-local-name="Romanian" class="interlanguage-link-target"><span>Română</span></a></li><li class="interlanguage-link interwiki-ru mw-list-item"><a href="https://ru.wikipedia.org/wiki/%D0%A0%D0%B0%D0%B1%D0%B4%D0%BE%D0%BC%D0%B8%D0%BE%D1%81%D0%B0%D1%80%D0%BA%D0%BE%D0%BC%D0%B0" title="Рабдомиосаркома – Russian" lang="ru" hreflang="ru" data-title="Рабдомиосаркома" data-language-autonym="Русский" data-language-local-name="Russian" class="interlanguage-link-target"><span>Русский</span></a></li><li class="interlanguage-link interwiki-simple mw-list-item"><a href="https://simple.wikipedia.org/wiki/Rhabdomyosarcoma" title="Rhabdomyosarcoma – Simple English" lang="en-simple" hreflang="en-simple" data-title="Rhabdomyosarcoma" data-language-autonym="Simple English" data-language-local-name="Simple English" class="interlanguage-link-target"><span>Simple English</span></a></li><li class="interlanguage-link interwiki-sr mw-list-item"><a href="https://sr.wikipedia.org/wiki/Rabdomiosarkom" title="Rabdomiosarkom – Serbian" lang="sr" hreflang="sr" data-title="Rabdomiosarkom" data-language-autonym="Српски / srpski" data-language-local-name="Serbian" class="interlanguage-link-target"><span>Српски / srpski</span></a></li><li class="interlanguage-link interwiki-sh mw-list-item"><a href="https://sh.wikipedia.org/wiki/Rabdomiosarkom" title="Rabdomiosarkom – Serbo-Croatian" lang="sh" hreflang="sh" data-title="Rabdomiosarkom" data-language-autonym="Srpskohrvatski / српскохрватски" data-language-local-name="Serbo-Croatian" class="interlanguage-link-target"><span>Srpskohrvatski / српскохрватски</span></a></li><li class="interlanguage-link interwiki-tt mw-list-item"><a href="https://tt.wikipedia.org/wiki/%D0%A0%D0%B0%D0%B1%D0%B4%D0%BE%D0%BC%D0%B8%D0%BE%D1%81%D0%B0%D1%80%D0%BA%D0%BE%D0%BC%D0%B0_(%D0%BA%D2%AF%D0%B7%D0%B4%D3%99)" title="Рабдомиосаркома (күздә) – Tatar" lang="tt" hreflang="tt" data-title="Рабдомиосаркома (күздә)" data-language-autonym="Татарча / tatarça" data-language-local-name="Tatar" class="interlanguage-link-target"><span>Татарча / tatarça</span></a></li><li class="interlanguage-link interwiki-tr mw-list-item"><a href="https://tr.wikipedia.org/wiki/Rabdomyosarkom" title="Rabdomyosarkom – Turkish" lang="tr" hreflang="tr" data-title="Rabdomyosarkom" data-language-autonym="Türkçe" data-language-local-name="Turkish" class="interlanguage-link-target"><span>Türkçe</span></a></li><li class="interlanguage-link interwiki-uk mw-list-item"><a href="https://uk.wikipedia.org/wiki/%D0%A0%D0%B0%D0%B1%D0%B4%D0%BE%D0%BC%D1%96%D0%BE%D1%81%D0%B0%D1%80%D0%BA%D0%BE%D0%BC%D0%B0" title="Рабдоміосаркома – Ukrainian" lang="uk" hreflang="uk" data-title="Рабдоміосаркома" data-language-autonym="Українська" data-language-local-name="Ukrainian" class="interlanguage-link-target"><span>Українська</span></a></li><li class="interlanguage-link interwiki-ur mw-list-item"><a href="https://ur.wikipedia.org/wiki/%D8%B9%D8%B5%D9%88%DB%8C_%D8%B9%D8%B6%D9%84%DB%8C_%D9%84%D8%AD%D9%85%D9%88%D9%85%DB%81" title="عصوی عضلی لحمومہ – Urdu" lang="ur" hreflang="ur" data-title="عصوی عضلی لحمومہ" data-language-autonym="اردو" data-language-local-name="Urdu" class="interlanguage-link-target"><span>اردو</span></a></li><li class="interlanguage-link interwiki-zh mw-list-item"><a href="https://zh.wikipedia.org/wiki/%E6%A8%AA%E7%BA%B9%E8%82%8C%E8%82%89%E7%98%A4" title="横纹肌肉瘤 – Chinese" lang="zh" hreflang="zh" data-title="横纹肌肉瘤" data-language-autonym="中文" data-language-local-name="Chinese" class="interlanguage-link-target"><span>中文</span></a></li> </ul> <div class="after-portlet after-portlet-lang"><span class="wb-langlinks-edit wb-langlinks-link"><a href="https://www.wikidata.org/wiki/Special:EntityPage/Q1898141#sitelinks-wikipedia" title="Edit interlanguage links" class="wbc-editpage">Edit links</a></span></div> </div> </div> </div> </header> <div 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searchaux" style="display:none">Cancer originating in precursors to muscle cells</div> <div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Medical condition</div><style data-mw-deduplicate="TemplateStyles:r1257001546">.mw-parser-output .infobox-subbox{padding:0;border:none;margin:-3px;width:auto;min-width:100%;font-size:100%;clear:none;float:none;background-color:transparent}.mw-parser-output .infobox-3cols-child{margin:auto}.mw-parser-output .infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox ib-medical-condition"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">Rhabdomyosarcoma</th></tr><tr style="background-color: #f8f9fa;"><td colspan="2" class="infobox-full-data"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:Rms1.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/5/56/Rms1.jpg/220px-Rms1.jpg" decoding="async" width="220" height="156" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/5/56/Rms1.jpg/330px-Rms1.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/5/56/Rms1.jpg/440px-Rms1.jpg 2x" data-file-width="882" data-file-height="627" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data"><a href="/wiki/Contrast_CT" title="Contrast CT">Non-contrast CT scan</a> of head showing a large mass without any intracranial extension. The diagnosis was <a href="/wiki/Post-auricular_muscle" class="mw-redirect" title="Post-auricular muscle">post-auricular</a> congenital <a href="/wiki/Alveolar_rhabdomyosarcoma" title="Alveolar rhabdomyosarcoma">alveolar rhabdomyosarcoma</a>.</td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="/wiki/Oncology" title="Oncology">Oncology</a></td></tr></tbody></table> <p><b>Rhabdomyosarcoma</b> (<b>RMS</b>) is a highly aggressive form of <a href="/wiki/Cancer" title="Cancer">cancer</a> that develops from <a href="/wiki/Mesenchymal_cell" class="mw-redirect" title="Mesenchymal cell">mesenchymal cells</a> that have failed to fully differentiate into <a href="/wiki/Myocyte" class="mw-redirect" title="Myocyte">myocytes</a> of <a href="/wiki/Skeletal_muscle" title="Skeletal muscle">skeletal muscle</a>. Cells of the <a href="/wiki/Neoplasm" title="Neoplasm">tumor</a> are identified as <a href="/wiki/Rhabdomyoblast" title="Rhabdomyoblast">rhabdomyoblasts</a>.<sup id="cite_ref-CO2021_1-0" class="reference"><a href="#cite_note-CO2021-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> </p><p>The four subtypes are <a href="/wiki/Embryonal_rhabdomyosarcoma" title="Embryonal rhabdomyosarcoma">embryonal rhabdomyosarcoma</a>, <a href="/wiki/Alveolar_rhabdomyosarcoma" title="Alveolar rhabdomyosarcoma">alveolar rhabdomyosarcoma</a>, <a href="/wiki/Pleomorphic_rhabdomyosarcoma" class="mw-redirect" title="Pleomorphic rhabdomyosarcoma">pleomorphic rhabdomyosarcoma</a>, and spindle-cell/sclerosing rhabdomyosarcoma.<sup id="cite_ref-WHO2020_2-0" class="reference"><a href="#cite_note-WHO2020-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Embryonal and alveolar are the main groups, and these types are the most common <a href="/wiki/Soft_tissue_sarcoma" class="mw-redirect" title="Soft tissue sarcoma">soft tissue sarcomas</a> of childhood and adolescence. The pleomorphic type is usually found in adults.<sup id="cite_ref-Robbins2018_3-0" class="reference"><a href="#cite_note-Robbins2018-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p><p>It is generally considered to be a disease of childhood, as the vast majority of cases occur in those below the age of 18. It is commonly described as one of the <a href="/wiki/Small-blue-round-cell_tumor" title="Small-blue-round-cell tumor">small-blue-round-cell tumors</a> of childhood due to its appearance on an <a href="/wiki/H%26E_stain" title="H&E stain">H&E stain</a>.<sup id="cite_ref-Chen_4-0" class="reference"><a href="#cite_note-Chen-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Despite being relatively rare, it accounts for approximately 40% of all recorded <a href="/wiki/Soft-tissue_sarcoma" title="Soft-tissue sarcoma">soft-tissue sarcomas</a>.<sup id="cite_ref-:0_5-0" class="reference"><a href="#cite_note-:0-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-6" class="reference"><a href="#cite_note-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-7" class="reference"><a href="#cite_note-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p><p>RMS can occur in any soft-tissue site in the body, but is primarily found in the head, neck, orbit, genitourinary tract, genitals, and extremities. No clear risk factors have been identified, but the disease has been associated with some congenital abnormalities.<sup id="cite_ref-:0_5-1" class="reference"><a href="#cite_note-:0-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:1_8-0" class="reference"><a href="#cite_note-:1-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> Signs and symptoms vary according to tumor site, and <a href="/wiki/Prognosis" title="Prognosis">prognosis</a> is closely tied to the location of the primary tumor. Common sites of <a href="/wiki/Metastasis" title="Metastasis">metastasis</a> include the lungs, bone marrow, and bones.<sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:7_10-0" class="reference"><a href="#cite_note-:7-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> There are many classification systems for RMS and a variety of defined histological types. Embryonal rhabdomyosarcoma is the most common type and comprises about 60% of cases.<sup id="cite_ref-:2_11-0" class="reference"><a href="#cite_note-:2-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> </p><p>Outcomes vary considerably, with five-year survival rates between 35 and 95%, depending on the type of RMS involved, so clear diagnosis is critical for effective treatment and management.<sup id="cite_ref-:2_11-1" class="reference"><a href="#cite_note-:2-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:3_12-0" class="reference"><a href="#cite_note-:3-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p><p>Treatment usually involves a combination of surgery, chemotherapy, and radiation. 60 to 70% of newly diagnosed patients with nonmetastatic disease can be cured using this combined approach to therapy. Despite aggressive multimodality treatment, less than 20% of patients with metastatic RMS are able to be cured of their disease.<sup id="cite_ref-13" class="reference"><a href="#cite_note-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Types">Types</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=1" title="Edit section: Types"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:Rms2.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/0/0c/Rms2.jpg/220px-Rms2.jpg" decoding="async" width="220" height="167" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/0/0c/Rms2.jpg/330px-Rms2.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/0/0c/Rms2.jpg/440px-Rms2.jpg 2x" data-file-width="763" data-file-height="579" /></a><figcaption>Photomicrograph showing nodules of tumor cells separated by hyalinised fibrous septae (50×, HE stain). Inset: Discohesive large tumor cells with hyperchromatic nucleus and scant cytoplasm (200×, HE stain). The diagnosis was postauricular congenital alveolar rhabdomyosarcoma.</figcaption></figure><p> Given the difficulty in diagnosing rhabdomyosarcoma, definitive classification of subtypes has proven difficult. As a result, classification systems vary by institute and organization. Rhabdomyosarcoma in the 2020 WHO classification, though, is listed as four <a href="/wiki/Histology" title="Histology">histological</a> subtypes: embryonal, alveolar, pleomorphic, and spindle-cell/sclerosing.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2021)">citation needed</span></a></i>]</sup> </p><div class="mw-heading mw-heading3"><h3 id="Embryonal">Embryonal</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=2" title="Edit section: Embryonal"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/Embryonal_rhabdomyosarcoma" title="Embryonal rhabdomyosarcoma">Embryonal rhabdomyosarcoma</a> (ERMS) is the most common histological variant, comprising about 60–70% of childhood cases. It is most common in children birth to four years old, with a maximum reported incidence of four cases per million children. ERMS is characterized by spindle-shaped cells with a stromal-rich appearance, and the morphology is similar to the developing muscle cells of a 6- to 8-week-old embryo. Tumors often present in the head and neck, as well as the genitourinary tract.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2021)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading4"><h4 id="Embryonal_subtype">Embryonal subtype</h4><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=3" title="Edit section: Embryonal subtype"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/Sarcoma_botryoides" title="Sarcoma botryoides">Botryoid rhabdomyosarcoma</a> is almost always found in mucosal-lined organs, including the vagina, bladder, and nasopharynx (although presentation in the nasopharynx typically affects older children). It often presents in infants younger than a year old, as a round, grape-like mass on the affected organ. Histologically, cells of the botryoid variant are defined by a dense tumor layer under an epithelium (cambium layer).<sup id="cite_ref-:4_14-0" class="reference"><a href="#cite_note-:4-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> This subtype has a good prognosis.<sup id="cite_ref-:2_11-2" class="reference"><a href="#cite_note-:2-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:3_12-1" class="reference"><a href="#cite_note-:3-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p><p>Botryoid rhabdomyosarcoma is also sometimes present in adult women, found in the cervix or uterus.<sup id="cite_ref-Li_15-0" class="reference"><a href="#cite_note-Li-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Alveolar">Alveolar</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=4" title="Edit section: Alveolar"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/Alveolar_rhabdomyosarcoma" title="Alveolar rhabdomyosarcoma">Alveolar rhabdomyosarcoma</a> (ARMS) is the second-most common type. ARMS comprises around 20–25% of RMS-related tumors, and it is equally distributed among all age groups with an incidence of about one case per million people ages 0 to 19. For this reason, it is the most common form of RMS observed in young adults and teenagers, who are less prone to the embryonal variant. This type of RMS is characterized by densely packed, round cells that arrange around spaces similar in shape to pulmonary alveoli, although variants have been discovered without these characteristic alveolar spacings. ARMS tends to form more often in the extremities, trunk, and peritoneum. It is also typically more aggressive than ERMS.<sup id="cite_ref-:3_12-2" class="reference"><a href="#cite_note-:3-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:4_14-1" class="reference"><a href="#cite_note-:4-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Pleomorphic">Pleomorphic</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=5" title="Edit section: Pleomorphic"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/Pleomorphic_rhabdomyosarcoma" class="mw-redirect" title="Pleomorphic rhabdomyosarcoma">Pleomorphic rhabdomyosarcoma</a> (undifferentiated rhabdomyosarcoma), also known as <a href="/wiki/Anaplasia" title="Anaplasia">anaplastic</a> rhabdomyosarcoma, is defined by the presence of pleomorphic cells with large, lobate hyperchromatic nuclei and multipolar mitotic figures. These tumors display high heterogeneity and extremely poor differentiation. The pleomorphic cells may be diffuse or localized, with the diffuse variation correlating to a worse prognosis.<sup id="cite_ref-Cecchetto_2561–2567_16-0" class="reference"><a href="#cite_note-Cecchetto_2561–2567-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> It occurs most often in adults, rarely in children, and is often discovered in the extremities.<sup id="cite_ref-:7_10-1" class="reference"><a href="#cite_note-:7-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-17" class="reference"><a href="#cite_note-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> Due to the lack of discernible separation among cancers of this type, clinicians often label undiagnosed sarcomas with little to no discernible features as anaplastic RMS. It is the most aggressive type of RMS, and often requires intensive treatment.<sup id="cite_ref-18" class="reference"><a href="#cite_note-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Spindle-cell/sclerosing"><span id="Spindle-cell.2Fsclerosing"></span>Spindle-cell/sclerosing</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=6" title="Edit section: Spindle-cell/sclerosing"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><b>Spindle-cell/sclerosing rhabdomyosarcoma</b> is an added subtype listed in the 2020 <a href="/wiki/WHO" class="mw-redirect" title="WHO">WHO</a> classification of soft-tissue sarcomas.<sup id="cite_ref-WHO2020_2-1" class="reference"><a href="#cite_note-WHO2020-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p><p>This subtype is very similar to that of <a href="/wiki/Leiomyosarcoma" title="Leiomyosarcoma">leiomyosarcoma</a> (cancer of the <a href="/wiki/Smooth_muscle_tissue" class="mw-redirect" title="Smooth muscle tissue">smooth muscle tissue</a>), and it has a fascicular, spindled, and leiomyomatous growth pattern with notable rhabdomyoblastic differentiation . It occurs most commonly in the paratesticular region, and the prognosis for this particular form of RMS is excellent with a reported <a href="/wiki/Five-year_survival_rate" title="Five-year survival rate">five-year survival rate</a> of 95%.<sup id="cite_ref-:3_12-3" class="reference"><a href="#cite_note-:3-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> The <a href="/wiki/Sclerosis_(medicine)" title="Sclerosis (medicine)">sclerosing aspect</a> of this subtype has a <a href="/wiki/Hyaline" title="Hyaline">hyaline</a> <a href="/wiki/Hyaline_arteriolosclerosis" class="mw-redirect" title="Hyaline arteriolosclerosis">sclerosis</a> and pseudovascular development.<sup id="cite_ref-19" class="reference"><a href="#cite_note-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup> </p><p>Multiple classification systems have been proposed for guiding management and treatment, and the most recent and widely used classification system is the "International Classification of Rhabdomyosarcoma" or ICR. It was created by the IRSG in 1995 after their series of four multi-institutional trials aimed at studying the presentation, histology, epidemiology, and treatment of RMS (IRSG I–IV).<sup id="cite_ref-:2_11-3" class="reference"><a href="#cite_note-:2-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> The ICR system is based on prognostic indicators identified in IRSG I–IV. Pleomorphic rhabdomyosarcoma usually occurs in adults rather than children, and is therefore not included in this system.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2021)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=7" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>RMS can occur in almost any soft-tissue site in the body; the most common primary sites are genitourinary (24%), parameningeal (16%), extremity (19%), orbit (9%), other head and neck (10%), and miscellaneous other sites (22%).<sup id="cite_ref-:4_14-2" class="reference"><a href="#cite_note-:4-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> RMS often presents as a mass, but signs and symptoms can vary widely depending on the site of the primary tumor. Genitourinary tumors may present with <a href="/wiki/Hematuria" title="Hematuria">hematuria</a>, urinary tract obstruction, and/or a scrotal or vaginal mass. Tumors that arise in the <a href="/wiki/Retroperitoneal_space" title="Retroperitoneal space">retroperitoneum</a> and mediastinum can become quite large before producing signs and symptoms. Parameningeal tumors may present with cranial nerve dysfunction, symptoms of sinusitis, ear discharge, headaches, and facial pain. Orbital tumors often present with orbital swelling and <a href="/wiki/Exophthalmos" title="Exophthalmos">proptosis</a>. Extremity tumors generally present as a rapidly enlarging, firm mass in the relevant tissue. The cancer's prevalence in the head, face, and neck will often allow for earlier signs of the disease simply due to the obvious nature of tumors in these locations.<sup id="cite_ref-:4_14-3" class="reference"><a href="#cite_note-:4-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> Despite the varying presentation and typically aggressive nature of the disease, RMS has the potential to be diagnosed and treated early. The fourth IRSG study found that 23% of patients were diagnosed in time for a complete resection of their cancer, and 15% had resection with only minimal remnants of the diseased cells.<sup id="cite_ref-20" class="reference"><a href="#cite_note-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Risk_factors">Risk factors</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=8" title="Edit section: Risk factors"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Rhabdomyosarcoma is difficult to diagnose. Risk factors that increase the likelihood of this cancer include inherited disorders such as <a href="/wiki/Li-Fraumeni_syndrome" class="mw-redirect" title="Li-Fraumeni syndrome">Li-Fraumeni syndrome</a>, <a href="/wiki/Neurofibromatosis_type_1" class="mw-redirect" title="Neurofibromatosis type 1">Neurofibromatosis type 1</a>, <a href="/wiki/Beckwith-Wiedemann_syndrome" class="mw-redirect" title="Beckwith-Wiedemann syndrome">Beckwith-Wiedemann syndrome</a>, <a href="/wiki/Costello_syndrome" title="Costello syndrome">Costello syndrome</a>, <a href="/wiki/Noonan_syndrome" title="Noonan syndrome">Noonan syndrome</a>,<sup id="cite_ref-CGOV2020_21-0" class="reference"><a href="#cite_note-CGOV2020-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup> and <a href="/wiki/Sertoli%E2%80%93Leydig_cell_tumour" title="Sertoli–Leydig cell tumour">DICER1 syndrome</a>.<sup id="cite_ref-Robertson_22-0" class="reference"><a href="#cite_note-Robertson-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Genetic">Genetic</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=9" title="Edit section: Genetic"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>There are multiple genetic lesions associated with rhabdomyosarcoma, but there has been little consistent data demonstrating an association between specific genetic abnormalities and outcome. However, alveolar and embryonal types of RMS can be distinguished cytogenetically, and identification of specific genetic lesions can allow for accurate classification of the ARMS subtype when the histopathological findings are equivocal or unclear. This is valuable for clinical practice as the alveolar type presents a higher risk to the patient and will often require more aggressive treatment than the embryonal type. Thus, ARMS is also referred to as <b>Fusion Positive</b> rhabdomyosarcoma (<b>FP-RMS</b>). Up to 90% of alveolar RMS cases present with a translocations of t(2;13)(q35, q14) or, less commonly, t(1;13)(p36, q15).<sup id="cite_ref-:9_23-0" class="reference"><a href="#cite_note-:9-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:12_24-0" class="reference"><a href="#cite_note-:12-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> Both involve the translocation of a DNA binding domain of either <i><a href="/wiki/PAX3" title="PAX3">PAX3</a></i><sup id="cite_ref-:12_24-1" class="reference"><a href="#cite_note-:12-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> or <i><a href="/wiki/PAX7" title="PAX7">PAX7</a></i><sup id="cite_ref-:9_23-1" class="reference"><a href="#cite_note-:9-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup><i>,</i> a member of the Paired Box family of transcription factors, to a transactivation site on <i><a href="/wiki/FOXO1" class="mw-redirect" title="FOXO1">FOXO1</a></i> (previously known as <i>FKHR)</i>, a member of the forkhead/HNF-3 transcription factor family.<sup id="cite_ref-:5_25-0" class="reference"><a href="#cite_note-:5-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup> The t(2;13) translocation results in a fusion of the <i><a href="/wiki/PAX3" title="PAX3">PAX3</a></i> gene with <i><a href="/wiki/FOXO1" class="mw-redirect" title="FOXO1">FOXO1</a></i>, while the t(1;13) translocation involves the fusion of <i><a href="/wiki/PAX7" title="PAX7">PAX7</a></i> with <i><a href="/wiki/FOXO1" class="mw-redirect" title="FOXO1">FOXO1</a></i>.<sup id="cite_ref-:6_26-0" class="reference"><a href="#cite_note-:6-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup> <i>PAX3</i> has a demonstrated role in muscle cell development, which supports its potential role in RMS. The t(2;13) translocation can result in the <i>PAX3-FKHR</i> fusion product, which is indicative of classic cystic ARMS.<sup id="cite_ref-:6_26-1" class="reference"><a href="#cite_note-:6-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup> Cases of FP-RMS are associated with a poorer prognosis than fusion-negative RMS.<sup id="cite_ref-27" class="reference"><a href="#cite_note-27"><span class="cite-bracket">[</span>27<span class="cite-bracket">]</span></a></sup> </p><p>The fusion protein presents a potential therapeutic target, and in recent years more research has been conducted to clarify the role of <i>PAX3-FOXO1</i> in FP-RMS. PAX3-FOXO1 is now known to drive key oncogenes such as MYC and MYCN by creating long-distance genetic interactions by <a href="/wiki/Super-enhancer" title="Super-enhancer">super enhancers</a>.<sup id="cite_ref-:13_28-0" class="reference"><a href="#cite_note-:13-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></sup> In this context, PAX3-FOXO1 both (1) drives the expression of <i>MYC</i>, <i>MYCN</i> and even <i>MYOD1</i> (a transcription factor highly expressed in all RMS subtypes) but also (2) co-binds with these <a href="/wiki/Transcription_factor" title="Transcription factor">master transcription factors</a> at super enhancers to support cancer growth.<sup id="cite_ref-:13_28-1" class="reference"><a href="#cite_note-:13-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></sup> Furthermore, it was demonstrated that FP-RMS subtypes were especially sensitive to inhibitors (such as <a href="/wiki/JQ1" title="JQ1">JQ1</a>) of a super enhancer bound protein <a href="/wiki/BRD4" title="BRD4">BRD4</a>.<sup id="cite_ref-:13_28-2" class="reference"><a href="#cite_note-:13-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></sup> </p><p>Embryonal RMS usually presents with a <a href="/wiki/Loss_of_heterozygosity" title="Loss of heterozygosity">loss of heterozygosity</a> (LOH) in the short arm of <a href="/wiki/Chromosome_11_(human)" class="mw-redirect" title="Chromosome 11 (human)">chromosome 11</a> (p11,15.5).<sup id="cite_ref-:5_25-1" class="reference"><a href="#cite_note-:5-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-29" class="reference"><a href="#cite_note-29"><span class="cite-bracket">[</span>29<span class="cite-bracket">]</span></a></sup> This region is associated with multiple <a href="/wiki/Oncogene" title="Oncogene">oncogenes</a>, and the potential loss-of-function of this region is likely associated with the loss of a tumor suppressor. However, the specific consequences of this LOH at (p11,15.5) have yet to be determined. The short arm of chromosome 11 is also the site of the insulin-like growth factor 2 gene (IGF-2), which is often over-expressed in RMS.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2021)">citation needed</span></a></i>]</sup> </p><p>The loss-of-function of <a href="/wiki/P53" title="P53">tumor suppressor p53</a> is associated with many cancers including rhabdomyosarcoma,<sup id="cite_ref-30" class="reference"><a href="#cite_note-30"><span class="cite-bracket">[</span>30<span class="cite-bracket">]</span></a></sup> and approximately 50% of RMS cases have been shown to carry some form of mutation to the P53 gene<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (May 2022)">citation needed</span></a></i>]</sup>. Other oncogenes often associated with rhabdomyosarcoma, albeit with less frequency, include <i><a href="/wiki/NMYC" class="mw-redirect" title="NMYC">NMYC</a></i>, <i><a href="/wiki/Neuroblastoma_RAS_viral_oncogene_homolog" title="Neuroblastoma RAS viral oncogene homolog">NRAS</a></i>, <i><a href="/wiki/KRAS" title="KRAS">KRAS</a></i>, <i><a href="/wiki/P16" title="P16">P16</a></i>, and c-<i><a href="/wiki/C-Met" class="mw-redirect" title="C-Met">Met</a>.</i><sup id="cite_ref-:5_25-2" class="reference"><a href="#cite_note-:5-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-31" class="reference"><a href="#cite_note-31"><span class="cite-bracket">[</span>31<span class="cite-bracket">]</span></a></sup> One study showed that 35% of embryonal RMS tumors contained activating mutations in either <i>NRAS</i> or <i>KRAS</i> and it is worth noting that <i>ras</i> activation has been shown to block myogenic differentiation, which could help explain its potential role in rhabdomyosarcogenesis.<sup id="cite_ref-32" class="reference"><a href="#cite_note-32"><span class="cite-bracket">[</span>32<span class="cite-bracket">]</span></a></sup> More recently, a mechanistic and <a href="/wiki/Epigenetics" title="Epigenetics">epigenetic</a> link between mutant RAS isoforms and a block of myogenic differentiation has been demonstrated.<sup id="cite_ref-:14_33-0" class="reference"><a href="#cite_note-:14-33"><span class="cite-bracket">[</span>33<span class="cite-bracket">]</span></a></sup> Furthermore, it has been shown that this differentiation block can be overcome with a clinical stage inhibitor of the <a href="/wiki/Mitogen-activated_protein_kinase" title="Mitogen-activated protein kinase">MAP kinase pathway</a> known as a <a href="/wiki/MEK_inhibitor" title="MEK inhibitor">MEK inhibitor</a>.<sup id="cite_ref-:14_33-1" class="reference"><a href="#cite_note-:14-33"><span class="cite-bracket">[</span>33<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=10" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Rhabdomyosarcoma is often difficult to diagnose due to its similarities to other cancers and varying levels of differentiation. It is loosely classified as one of the <a href="/wiki/Small-blue-round-cell_tumor" title="Small-blue-round-cell tumor">small-blue-round-cell tumors</a> due to its appearance on an H&E stain. Other cancers that share this classification include <a href="/wiki/Neuroblastoma" title="Neuroblastoma">neuroblastoma</a>, <a href="/wiki/Ewing_sarcoma" title="Ewing sarcoma">Ewing sarcoma</a>, and <a href="/wiki/Lymphoma" title="Lymphoma">lymphoma</a>, and a diagnosis of RMS requires confident elimination of these morphologically similar diseases.<sup id="cite_ref-:4_14-4" class="reference"><a href="#cite_note-:4-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> The defining diagnostic trait for RMS is confirmation of malignant skeletal muscle differentiation with myogenesis (presenting as a plump, pink cytoplasm) under <a href="/wiki/Microscopy" title="Microscopy">light microscopy</a>.<sup id="cite_ref-:0_5-2" class="reference"><a href="#cite_note-:0-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> Cross striations may or may not be present. Accurate diagnosis is usually accomplished through immunohistochemical staining for muscle-specific proteins such as <a href="/wiki/Myogenin" title="Myogenin">myogenin</a>, muscle-specific <a href="/wiki/Actin" title="Actin">actin</a>, <a href="/wiki/Desmin" title="Desmin">desmin</a>, <a href="/wiki/Myosin" title="Myosin">D-myosin</a>, and <a href="/wiki/MyoD" title="MyoD">myoD</a>1.<sup id="cite_ref-:5_25-3" class="reference"><a href="#cite_note-:5-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:11_34-0" class="reference"><a href="#cite_note-:11-34"><span class="cite-bracket">[</span>34<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-35" class="reference"><a href="#cite_note-35"><span class="cite-bracket">[</span>35<span class="cite-bracket">]</span></a></sup> Myogenin, in particular, has been shown to be highly specific to RMS,<sup id="cite_ref-36" class="reference"><a href="#cite_note-36"><span class="cite-bracket">[</span>36<span class="cite-bracket">]</span></a></sup> although the diagnostic significance of each protein marker may vary depending on the type and location of the malignant cells. The alveolar type of RMS tends to have stronger muscle-specific protein staining. Electron microscopy may also aid in diagnosis, with the presence of actin and myosin or <a href="/wiki/Sarcomere#Bands" title="Sarcomere">Z bands</a> pointing to a positive diagnosis of RMS.<sup id="cite_ref-:0_5-3" class="reference"><a href="#cite_note-:0-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:11_34-1" class="reference"><a href="#cite_note-:11-34"><span class="cite-bracket">[</span>34<span class="cite-bracket">]</span></a></sup> Classification into types and subtypes is accomplished through further analysis of cellular morphology (alveolar spacings, presence of cambium layer, aneuploidy, etc.) as well as genetic sequencing of tumor cells. Some genetic markers, such as the <i>PAX3-FKHR</i> fusion gene expression in alveolar RMS, can aid in diagnosis. <a href="/wiki/Open_biopsy" title="Open biopsy">Open biopsy</a> is usually required to obtain sufficient tissue for accurate diagnosis. All findings must be considered in context, as no one trait is a definitive indicator for RMS.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2020)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading3"><h3 id="Staging">Staging</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=11" title="Edit section: Staging"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Following diagnosis and histopathological analysis, various <a href="/wiki/Medical_imaging" title="Medical imaging">imaging techniques</a> may be used, including <a href="/wiki/MRI" class="mw-redirect" title="MRI">MRI</a>, <a href="/wiki/Medical_ultrasound" title="Medical ultrasound">ultrasound</a>, and a <a href="/wiki/Bone_scintigraphy" title="Bone scintigraphy">bone scan</a> in order to determine the extent of local invasion and any metastasis. Further investigational techniques may be necessary depending on tumor sites. A <a href="/wiki/Meninges" title="Meninges">parameningeal</a> presentation of RMS will often require a <a href="/wiki/Lumbar_puncture" title="Lumbar puncture">lumbar puncture</a> to rule out metastasis to the meninges. A paratesticular presentation will often require an abdominal CT to rule out local lymph node involvement, and so on. Outcomes are strongly tied to the extent of the disease, and its early mapping is important for treatment planning.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2021)">citation needed</span></a></i>]</sup> </p><p>The current staging system for rhabdomyosarcoma is unusual relative to most cancers. It utilizes a modified <a href="/wiki/TNM_staging_system" title="TNM staging system">TNM</a> (tumor-nodes-metastasis) system originally developed by the IRSG.<sup id="cite_ref-:2_11-4" class="reference"><a href="#cite_note-:2-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:3_12-4" class="reference"><a href="#cite_note-:3-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:10_37-0" class="reference"><a href="#cite_note-:10-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> This system accounts for tumor size (> or <5 cm), lymph node involvement, tumor site, and presence of metastasis.<sup id="cite_ref-:4_14-5" class="reference"><a href="#cite_note-:4-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:10_37-1" class="reference"><a href="#cite_note-:10-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> It grades on a scale of 1 to 4 based on these criteria. In addition, patients are sorted by clinical group (from the clinical groups from the IRSG studies) based on the success of their first surgical resection.<sup id="cite_ref-:10_37-2" class="reference"><a href="#cite_note-:10-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> The current Children's Oncology Group protocols for the treatment of RMS categorize patients into one of four risk categories based on tumor grade and clinical group, and these risk categories have been shown to be highly predictive of outcome.<sup id="cite_ref-:11_34-2" class="reference"><a href="#cite_note-:11-34"><span class="cite-bracket">[</span>34<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-38" class="reference"><a href="#cite_note-38"><span class="cite-bracket">[</span>38<span class="cite-bracket">]</span></a></sup> </p> <table class="wikitable sortable"> <tbody><tr> <th>Tumor site </th> <th>Risk classification </th></tr> <tr> <td>Head and neck (orbit), biliary tract, genitourinary (excluding bladder and prostate) </td> <td>Favorable </td></tr> <tr> <td>Cranial parameningial, bladder, extremities, prostate, other </td> <td>Unfavorable </td></tr></tbody></table> <div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=12" title="Edit section: Treatment"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1251242444">.mw-parser-output .ambox{border:1px solid #a2a9b1;border-left:10px solid #36c;background-color:#fbfbfb;box-sizing:border-box}.mw-parser-output .ambox+link+.ambox,.mw-parser-output .ambox+link+style+.ambox,.mw-parser-output .ambox+link+link+.ambox,.mw-parser-output .ambox+.mw-empty-elt+link+.ambox,.mw-parser-output .ambox+.mw-empty-elt+link+style+.ambox,.mw-parser-output .ambox+.mw-empty-elt+link+link+.ambox{margin-top:-1px}html body.mediawiki .mw-parser-output .ambox.mbox-small-left{margin:4px 1em 4px 0;overflow:hidden;width:238px;border-collapse:collapse;font-size:88%;line-height:1.25em}.mw-parser-output .ambox-speedy{border-left:10px solid #b32424;background-color:#fee7e6}.mw-parser-output .ambox-delete{border-left:10px solid #b32424}.mw-parser-output .ambox-content{border-left:10px solid #f28500}.mw-parser-output .ambox-style{border-left:10px solid #fc3}.mw-parser-output .ambox-move{border-left:10px solid #9932cc}.mw-parser-output .ambox-protection{border-left:10px solid #a2a9b1}.mw-parser-output .ambox .mbox-text{border:none;padding:0.25em 0.5em;width:100%}.mw-parser-output .ambox .mbox-image{border:none;padding:2px 0 2px 0.5em;text-align:center}.mw-parser-output .ambox .mbox-imageright{border:none;padding:2px 0.5em 2px 0;text-align:center}.mw-parser-output .ambox .mbox-empty-cell{border:none;padding:0;width:1px}.mw-parser-output .ambox .mbox-image-div{width:52px}@media(min-width:720px){.mw-parser-output .ambox{margin:0 10%}}@media print{body.ns-0 .mw-parser-output .ambox{display:none!important}}</style><table class="box-Update plainlinks metadata ambox ambox-content ambox-Update" role="presentation"><tbody><tr><td class="mbox-image"><div class="mbox-image-div"><span typeof="mw:File"><span><img alt="" src="//upload.wikimedia.org/wikipedia/commons/thumb/5/53/Ambox_current_red_Americas.svg/42px-Ambox_current_red_Americas.svg.png" decoding="async" width="42" height="34" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/5/53/Ambox_current_red_Americas.svg/63px-Ambox_current_red_Americas.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/5/53/Ambox_current_red_Americas.svg/84px-Ambox_current_red_Americas.svg.png 2x" data-file-width="360" data-file-height="290" /></span></span></div></td><td class="mbox-text"><div class="mbox-text-span">This section needs to be <b>updated</b>. The reason given is: <a rel="nofollow" class="external free" href="https://www.worldcommunitygrid.org/about_us/article.s?articleId=777">https://www.worldcommunitygrid.org/about_us/article.s?articleId=777</a>.<span class="hide-when-compact"> Please help update this article to reflect recent events or newly available information.</span> <span class="date-container"><i>(<span class="date">August 2023</span>)</i></span></div></td></tr></tbody></table> <p>Treatment of rhabdomyosarcoma is a multidisciplinary practice involving the use of surgery, chemotherapy, radiation, and possibly <a href="/wiki/Cancer_immunotherapy" title="Cancer immunotherapy">immunotherapy</a>. Surgery is generally the first step in a combined therapeutic approach. Resectability varies depending on tumor site, and RMS often presents in sites that don't allow for full surgical resection without significant morbidity and loss of function. Less than 20% of RMS tumors are fully resected with negative margins. Rhabdomyosarcomas are highly chemosensitive, with approximately 80% of cases responding to chemotherapy. In fact, multi-agent chemotherapy is indicated for all patients with rhabdomyosarcoma. Before the use of adjuvant and neoadjuvant therapy involving chemotherapeutic agents, treatment solely by surgical means had a survival rate of <20%. Modern survival rates with adjuvant therapy are approximately 60–70%.<sup id="cite_ref-:1_8-1" class="reference"><a href="#cite_note-:1-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-39" class="reference"><a href="#cite_note-39"><span class="cite-bracket">[</span>39<span class="cite-bracket">]</span></a></sup> </p><p>There are two main methods of <a href="/wiki/Chemotherapy" title="Chemotherapy">chemotherapy</a> treatment for RMS. There is the <a href="/w/index.php?title=VAC_regimen&action=edit&redlink=1" class="new" title="VAC regimen (page does not exist)">VAC regimen</a>, consisting of <a href="/wiki/Vincristine" title="Vincristine">vincristine</a>, <a href="/wiki/Dactinomycin" title="Dactinomycin">actinomycin D</a>, and <a href="/wiki/Cyclophosphamide" title="Cyclophosphamide">cyclophosphamide</a>, and the IVA regimen, consisting of <a href="/wiki/Ifosfamide" title="Ifosfamide">ifosfamide</a>, vincristine, and actinomycin D. These drugs are administered in 9–15 cycles depending on the staging of the disease and other therapies used.<sup id="cite_ref-:11_34-3" class="reference"><a href="#cite_note-:11-34"><span class="cite-bracket">[</span>34<span class="cite-bracket">]</span></a></sup> Other drug and therapy combinations may also show additional benefit. Addition of <a href="/wiki/Doxorubicin" title="Doxorubicin">doxorubicin</a> and <a href="/wiki/Cisplatin" title="Cisplatin">cisplatin</a> to the VAC regimen was shown to increase survival rates of patients with alveolar-type, early-stage RMS in IRS study III, and this same addition improved survival rates and doubled bladder salvage rates in patients with stage III RMS of the bladder.<sup id="cite_ref-Cecchetto_2561–2567_16-1" class="reference"><a href="#cite_note-Cecchetto_2561–2567-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:11_34-4" class="reference"><a href="#cite_note-:11-34"><span class="cite-bracket">[</span>34<span class="cite-bracket">]</span></a></sup> In children and young adults with stage IV metastatic rhabdomyoscarcoma, a Cochrane review has found no evidence to support the use of high-dose chemotherapy as a standard therapy.<sup id="cite_ref-40" class="reference"><a href="#cite_note-40"><span class="cite-bracket">[</span>40<span class="cite-bracket">]</span></a></sup> </p><p><a href="/wiki/Radiation_therapy" title="Radiation therapy">Radiation therapy</a>, which kill cancer cells with focused doses of radiation, is often indicated in the treatment of rhabdomyosarcoma, and the exclusion of this treatment from disease management has been shown to increase recurrence rates. Radiation therapy is used when resecting the entirety of the tumor would involve disfigurement or loss of important organs (eye, bladder, etc.). Generally, in any case where a lack of complete resection is suspected, radiation therapy is indicated.<sup id="cite_ref-:4_14-6" class="reference"><a href="#cite_note-:4-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> Administration is usually following 6–12 weeks of chemotherapy if tumor cells are still present. The exception to this schedule is the presence of parameningeal tumors that have invaded the brain, spinal cord, or skull. In these cases radiation treatment is started immediately.<sup id="cite_ref-41" class="reference"><a href="#cite_note-41"><span class="cite-bracket">[</span>41<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-42" class="reference"><a href="#cite_note-42"><span class="cite-bracket">[</span>42<span class="cite-bracket">]</span></a></sup> In some cases, special radiation treatment may be required. Brachytherapy, or the placement of small, radioactive "seeds" directly inside the tumor or cancer site, is often indicated in children with tumors of sensitive areas such as the testicles, bladder, or vagina. This reduces scattering and the degree of late toxicity following dosing.<sup id="cite_ref-43" class="reference"><a href="#cite_note-43"><span class="cite-bracket">[</span>43<span class="cite-bracket">]</span></a></sup> Radiation therapy is more often indicated in higher stage classifications. </p><p><a href="/wiki/Immunotherapy" title="Immunotherapy">Immunotherapy</a> is a more recent treatment modality that is still in development. This method involves recruiting and training the patient's immune system to target the cancer cells. This can be accomplished through administering small molecules designed to pull immune cells towards the tumors, taking immune cells pulled from the patient and training to attack tumors through presentation with tumor antigen, or other experimental methods. A specific example here would be presenting some of the patient's dendritic cells, which direct the immune system to foreign cells, with the PAX3-FKHR fusion protein in order to focus the patient's immune system to the malignant RMS cells<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2020)">citation needed</span></a></i>]</sup>. All cancers, including rhabdomyosarcoma, could potentially benefit from this new, immune-based approach<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2020)">citation needed</span></a></i>]</sup>. </p> <div class="mw-heading mw-heading2"><h2 id="Prognostic">Prognostic</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=13" title="Edit section: Prognostic"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Prognosis in rhabdomyosarcoma patients has been shown to be dependent on age, tumor site, resectability of tumor, tumor size, regional <a href="/wiki/Lymph_node" title="Lymph node">lymph node involvement</a>, presence of metastasis, site and extent of metastasis, and biological and histopathological characteristics of the tumor cells.<sup id="cite_ref-:8_44-0" class="reference"><a href="#cite_note-:8-44"><span class="cite-bracket">[</span>44<span class="cite-bracket">]</span></a></sup> Survival after recurrence is poor, and new salvage therapy strategies are needed.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2022)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading2"><h2 id="Epidemiology">Epidemiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=14" title="Edit section: Epidemiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Rhabdomyosarcoma is the most common soft-tissue <a href="/wiki/Sarcoma" title="Sarcoma">sarcoma</a> in children as well as the third most common solid tumor in children. Recent estimates place the incidence of the disease at approximately 4.5 case per 1 million children/adolescents with approximately 250 new cases in the United States each year.<sup id="cite_ref-45" class="reference"><a href="#cite_note-45"><span class="cite-bracket">[</span>45<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-:8_44-1" class="reference"><a href="#cite_note-:8-44"><span class="cite-bracket">[</span>44<span class="cite-bracket">]</span></a></sup> With the vast majority of cases of RMS occurring in children or adolescents, two-thirds of reported cases occur in youths under the age of 10.<sup id="cite_ref-:0_5-4" class="reference"><a href="#cite_note-:0-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> RMS also occurs slightly more often in males than in females, with a ratio of approximately 1.3–1.5:1. In addition, slightly lower prevalence of the disease has been reported in black and Asian children relative to white children.<sup id="cite_ref-46" class="reference"><a href="#cite_note-46"><span class="cite-bracket">[</span>46<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-47" class="reference"><a href="#cite_note-47"><span class="cite-bracket">[</span>47<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-48" class="reference"><a href="#cite_note-48"><span class="cite-bracket">[</span>48<span class="cite-bracket">]</span></a></sup> In most cases, there are no clear predisposing risk factors for the development of RMS. It tends to occur sporadically with no obvious cause. However, RMS has been correlated with familial cancer syndromes and congenital abnormalities including <a href="/wiki/Neurofibromatosis_type_I" title="Neurofibromatosis type I">neurofibromatosis type 1</a>,<sup id="cite_ref-49" class="reference"><a href="#cite_note-49"><span class="cite-bracket">[</span>49<span class="cite-bracket">]</span></a></sup> <a href="/wiki/Beckwith%E2%80%93Wiedemann_syndrome" title="Beckwith–Wiedemann syndrome">Beckwith-Wiedemann syndrome</a>,<sup id="cite_ref-50" class="reference"><a href="#cite_note-50"><span class="cite-bracket">[</span>50<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-51" class="reference"><a href="#cite_note-51"><span class="cite-bracket">[</span>51<span class="cite-bracket">]</span></a></sup> <a href="/wiki/Li%E2%80%93Fraumeni_syndrome" title="Li–Fraumeni syndrome">Li–Fraumeni syndrome</a>,<sup id="cite_ref-52" class="reference"><a href="#cite_note-52"><span class="cite-bracket">[</span>52<span class="cite-bracket">]</span></a></sup> <a href="/wiki/Cardiofaciocutaneous_syndrome" title="Cardiofaciocutaneous syndrome">cardio-facio-cutaneous syndrome</a>,<sup id="cite_ref-53" class="reference"><a href="#cite_note-53"><span class="cite-bracket">[</span>53<span class="cite-bracket">]</span></a></sup> and <a href="/wiki/Costello_syndrome" title="Costello syndrome">Costello syndrome</a>.<sup id="cite_ref-54" class="reference"><a href="#cite_note-54"><span class="cite-bracket">[</span>54<span class="cite-bracket">]</span></a></sup> It has also been associated with parental use of cocaine and marijuana.<sup id="cite_ref-55" class="reference"><a href="#cite_note-55"><span class="cite-bracket">[</span>55<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="History">History</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=15" title="Edit section: History"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Rhabdomyosarcoma was first described by Weber, a German physician, in 1845,<sup id="cite_ref-56" class="reference"><a href="#cite_note-56"><span class="cite-bracket">[</span>56<span class="cite-bracket">]</span></a></sup> but it was not until the paper by Arthur Stout in 1946 that RMS was formally classified.<sup id="cite_ref-57" class="reference"><a href="#cite_note-57"><span class="cite-bracket">[</span>57<span class="cite-bracket">]</span></a></sup> The first thirty years of investigation were conducted by the Intergroup Rhabdomyosarcoma Study Group (IRSG), an independent National Cancer Institute (NCI)-funded cooperative that has become a part of the Children's Oncology Group.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2020)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading2"><h2 id="Research">Research</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=16" title="Edit section: Research"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/Cancer_stem_cells" class="mw-redirect" title="Cancer stem cells">Cancer stem cells</a> of rhabdomyosarcoma have been identified and <a href="/wiki/Fibroblast_growth_factor_receptor_3" title="Fibroblast growth factor receptor 3">fibroblast growth factor receptor 3</a> has been suggested as their marker. Preclinical animal studies that try to use conditionally replicating <a href="/wiki/Adenoviruses" class="mw-redirect" title="Adenoviruses">adenoviruses</a> against such cells are in progress.<sup id="cite_ref-pmid_=_24467821_58-0" class="reference"><a href="#cite_note-pmid_=_24467821-58"><span class="cite-bracket">[</span>58<span class="cite-bracket">]</span></a></sup> Epigenetic therapy for rhabdomyosarcoma is becoming more important.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2020)">citation needed</span></a></i>]</sup> A recent study by Bharathy et al. found that deacetylase inhibitor, entinostat works in aggressive subtype, alveolar rhabdomyosarcoma (aRMS) by specifically blocking the activity of HDAC3, thereby preventing epigenetic suppression of a microRNA that inhibits PAX3:FOXO1 translation. These findings and ongoing clinical trials (ADVL1513) shows promise for an effective therapy for some patients with aRMS.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2020)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading2"><h2 id="See_also">See also</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=17" title="Edit section: See also"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a href="/wiki/Rhabdomyoma" title="Rhabdomyoma">Rhabdomyoma</a></li></ul> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Rhabdomyosarcoma&action=edit&section=18" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist"> <div class="mw-references-wrap mw-references-columns"><ol class="references"> <li id="cite_note-CO2021-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-CO2021_1-0">^</a></b></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">.mw-parser-output cite.citation{font-style:inherit;word-wrap:break-word}.mw-parser-output .citation q{quotes:"\"""\"""'""'"}.mw-parser-output .citation:target{background-color:rgba(0,127,255,0.133)}.mw-parser-output .id-lock-free.id-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://www.cancer.org/cancer/rhabdomyosarcoma/about/what-is-rhabdomyosarcoma.html">"What Is Rhabdomyosarcoma?"</a>. <i>www.cancer.org</i>. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20200917092853/https://www.cancer.org/cancer/rhabdomyosarcoma/about/what-is-rhabdomyosarcoma.html">Archived</a> from the original on 2020-09-17<span class="reference-accessdate">. Retrieved <span class="nowrap">2021-02-11</span></span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=www.cancer.org&rft.atitle=What+Is+Rhabdomyosarcoma%3F&rft_id=https%3A%2F%2Fwww.cancer.org%2Fcancer%2Frhabdomyosarcoma%2Fabout%2Fwhat-is-rhabdomyosarcoma.html&rfr_id=info%3Asid%2Fen.wikipedia.org%3ARhabdomyosarcoma" class="Z3988"></span></span> </li> <li id="cite_note-WHO2020-2"><span class="mw-cite-backlink">^ <a href="#cite_ref-WHO2020_2-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-WHO2020_2-1"><sup><i><b>b</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFKallenHornick2021" class="citation journal cs1">Kallen, ME; Hornick, JL (January 2021). 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Retrieved <span class="nowrap">2020-03-29</span></span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Cancer+Causes+%26+Control&rft.atitle=Parents%27+use+of+cocaine+and+marijuana+and+increased+risk+of+rhabdomyosarcoma+in+their+children&rft.volume=4&rft.issue=3&rft.pages=217-224&rft.date=1993-05-01&rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A26153806%23id-name%3DS2CID&rft_id=info%3Adoi%2F10.1007%2Fbf00051316&rft.issn=0957-5243&rft_id=https%3A%2F%2Fwww.jstor.org%2Fstable%2F3552846%23id-name%3DJSTOR&rft_id=info%3Apmid%2F8318638&rft.aulast=Grufferman&rft.aufirst=S.&rft.au=Schwartz%2C+A.+G.&rft.au=Ruymann%2C+F.+B.&rft.au=Maurer%2C+H.+M.&rft_id=https%3A%2F%2Flink.springer.com%2Farticle%2F10.1007%2FBF00051316&rfr_id=info%3Asid%2Fen.wikipedia.org%3ARhabdomyosarcoma" class="Z3988"></span></span> </li> <li id="cite_note-56"><span class="mw-cite-backlink"><b><a href="#cite_ref-56">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFWeber1854" class="citation journal cs1">Weber, CO (1854). <a rel="nofollow" class="external text" href="https://zenodo.org/record/1575207">"Anatomische Untersuchung einer hypertrophischen Zunge nebst Bemerkungen über die Neubildung quergestreifter Muskelfasern"</a>. <i>Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin</i>. <b>7</b>: 115–125. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1007%2FBF01936232">10.1007/BF01936232</a>. <a href="/wiki/S2CID_(identifier)" class="mw-redirect" title="S2CID (identifier)">S2CID</a> <a rel="nofollow" class="external text" href="https://api.semanticscholar.org/CorpusID:34545149">34545149</a>. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20221114053656/https://zenodo.org/record/1575207">Archived</a> from the original on 2022-11-14<span class="reference-accessdate">. Retrieved <span class="nowrap">2022-11-14</span></span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Archiv+f%C3%BCr+Pathologische+Anatomie+und+Physiologie+und+f%C3%BCr+Klinische+Medicin&rft.atitle=Anatomische+Untersuchung+einer+hypertrophischen+Zunge+nebst+Bemerkungen+%C3%BCber+die+Neubildung+quergestreifter+Muskelfasern&rft.volume=7&rft.pages=115-125&rft.date=1854&rft_id=info%3Adoi%2F10.1007%2FBF01936232&rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A34545149%23id-name%3DS2CID&rft.aulast=Weber&rft.aufirst=CO&rft_id=https%3A%2F%2Fzenodo.org%2Frecord%2F1575207&rfr_id=info%3Asid%2Fen.wikipedia.org%3ARhabdomyosarcoma" class="Z3988"></span></span> </li> <li id="cite_note-57"><span class="mw-cite-backlink"><b><a href="#cite_ref-57">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFStout1946" class="citation journal cs1">Stout, Arthur Purdy (1946-03-01). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1803493">"Rhabdomyosarcoma of the Skeletal Muscles"</a>. <i>Annals of Surgery</i>. <b>123</b> (3): 447–472. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1097%2F00000658-194603000-00011">10.1097/00000658-194603000-00011</a>. <a href="/wiki/ISSN_(identifier)" class="mw-redirect" title="ISSN (identifier)">ISSN</a> <a rel="nofollow" class="external text" href="https://search.worldcat.org/issn/0003-4932">0003-4932</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1803493">1803493</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/17858752">17858752</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Annals+of+Surgery&rft.atitle=Rhabdomyosarcoma+of+the+Skeletal+Muscles&rft.volume=123&rft.issue=3&rft.pages=447-472&rft.date=1946-03-01&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC1803493%23id-name%3DPMC&rft.issn=0003-4932&rft_id=info%3Apmid%2F17858752&rft_id=info%3Adoi%2F10.1097%2F00000658-194603000-00011&rft.aulast=Stout&rft.aufirst=Arthur+Purdy&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC1803493&rfr_id=info%3Asid%2Fen.wikipedia.org%3ARhabdomyosarcoma" class="Z3988"></span></span> </li> <li id="cite_note-pmid_=_24467821-58"><span class="mw-cite-backlink"><b><a href="#cite_ref-pmid_=_24467821_58-0">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFTanoue_K2014" class="citation journal cs1">Tanoue K (Jan 2014). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3925355">"Survivin-responsive conditionally replicating adenovirus kills rhabdomyosarcoma stem cells more efficiently than their progeny"</a>. <i>J Transl Med</i>. <b>12</b>: 27. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1186%2F1479-5876-12-27">10.1186/1479-5876-12-27</a></span>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3925355">3925355</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/24467821">24467821</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=J+Transl+Med&rft.atitle=Survivin-responsive+conditionally+replicating+adenovirus+kills+rhabdomyosarcoma+stem+cells+more+efficiently+than+their+progeny.&rft.volume=12&rft.pages=27&rft.date=2014-01&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3925355%23id-name%3DPMC&rft_id=info%3Apmid%2F24467821&rft_id=info%3Adoi%2F10.1186%2F1479-5876-12-27&rft.au=Tanoue+K&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3925355&rfr_id=info%3Asid%2Fen.wikipedia.org%3ARhabdomyosarcoma" class="Z3988"></span></span> </li> </ol></div></div> <ul><li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFSabooKrajewskiZukotynskiHoward2012" class="citation journal cs1">Saboo, S. S.; Krajewski, K. M.; Zukotynski, K.; Howard, S.; Jagannathan, J. P.; Hornick, J. L.; Ramaiya, N. (2012). "Imaging Features of Primary and Secondary Adult Rhabdomyosarcoma". <i>American Journal of Roentgenology</i>. <b>199</b> (6): W694–W703. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.2214%2FAJR.11.8213">10.2214/AJR.11.8213</a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/23169742">23169742</a>.</cite><span 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dd:first-child::before,.mw-parser-output .hlist dt dt:first-child::before,.mw-parser-output .hlist dt li:first-child::before,.mw-parser-output .hlist li dd:first-child::before,.mw-parser-output .hlist li dt:first-child::before,.mw-parser-output .hlist li li:first-child::before{content:" (";font-weight:normal}.mw-parser-output .hlist dd dd:last-child::after,.mw-parser-output .hlist dd dt:last-child::after,.mw-parser-output .hlist dd li:last-child::after,.mw-parser-output .hlist dt dd:last-child::after,.mw-parser-output .hlist dt dt:last-child::after,.mw-parser-output .hlist dt li:last-child::after,.mw-parser-output .hlist li dd:last-child::after,.mw-parser-output .hlist li dt:last-child::after,.mw-parser-output .hlist li li:last-child::after{content:")";font-weight:normal}.mw-parser-output .hlist ol{counter-reset:listitem}.mw-parser-output .hlist ol>li{counter-increment:listitem}.mw-parser-output .hlist ol>li::before{content:" "counter(listitem)"\a0 "}.mw-parser-output .hlist dd ol>li:first-child::before,.mw-parser-output .hlist dt ol>li:first-child::before,.mw-parser-output .hlist li ol>li:first-child::before{content:" ("counter(listitem)"\a0 "}</style><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"></div><div role="navigation" class="navbox" aria-label="Navbox" style="width:100%; margin:0.5em 0 0.5em 0;;padding:3px"><table class="nowraplinks navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q1898141" class="extiw" title="d:Q1898141">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/ICD-10" title="ICD-10">10</a></b>: <a rel="nofollow" class="external text" href="https://icd.who.int/browse10/2019/en#/C49">C49</a>.M50</li><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/List_of_ICD-9_codes" title="List of ICD-9 codes">9-CM</a></b>: <a rel="nofollow" class="external text" href="http://www.icd9data.com/getICD9Code.ashx?icd9=171.9">171.9</a></li><li><b><a href="/wiki/International_Classification_of_Diseases_for_Oncology" title="International Classification of Diseases for Oncology">ICD-O</a></b>: <a rel="nofollow" class="external text" href="https://progenetix.org/subsets/?term_id=icdom:8900_3">M8900/3</a>–<a rel="nofollow" class="external text" href="https://progenetix.org/subsets/?term_id=icdom:8920_3">M8920/3</a></li><li><b><a href="/wiki/Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">OMIM</a></b>: <a rel="nofollow" class="external text" href="https://omim.org/entry/268210">268210</a></li><li><b><a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=D012208">D012208</a></li><li><b><a href="/wiki/Diseases_Database" title="Diseases Database">DiseasesDB</a></b>: <a rel="nofollow" class="external text" href="http://www.diseasesdatabase.com/ddb11485.htm">11485</a></li><li><b><a href="/wiki/SNOMED_CT" title="SNOMED CT">SNOMED CT</a></b>: <a rel="nofollow" class="external text" href="http://snomed.info/id/30924005">30924005</a></li></ul></div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">External resources</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus</a></b>: <a rel="nofollow" class="external text" href="https://www.nlm.nih.gov/medlineplus/ency/article/001429.htm">001429</a></li><li><b><a href="/wiki/EMedicine" title="EMedicine">eMedicine</a></b>: <a rel="nofollow" class="external text" href="https://emedicine.medscape.com/ent/641-overview">ent/641</a> <a rel="nofollow" class="external text" href="https://www.emedicine.com/ped/topic2005.htm#">ped/2005</a></li><li><b><a href="/wiki/Patient_UK" title="Patient UK">Patient UK</a></b>: <a rel="nofollow" class="external text" href="https://patient.info/doctor/Rhabdomyosarcoma">Rhabdomyosarcoma</a></li><li><b><a href="/wiki/Orphanet" title="Orphanet">Orphanet</a></b>: <a rel="nofollow" class="external text" href="https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=780">780</a></li></ul></div></div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Connective/soft_tissue_tumors_and_sarcomas" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><style data-mw-deduplicate="TemplateStyles:r1239400231">.mw-parser-output .navbar{display:inline;font-size:88%;font-weight:normal}.mw-parser-output .navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output .navbar-brackets::after{margin-left:-0.125em;content:" ]"}.mw-parser-output .navbar li{word-spacing:-0.125em}.mw-parser-output .navbar a>span,.mw-parser-output .navbar a>abbr{text-decoration:inherit}.mw-parser-output .navbar-mini abbr{font-variant:small-caps;border-bottom:none;text-decoration:none;cursor:inherit}.mw-parser-output .navbar-ct-full{font-size:114%;margin:0 7em}.mw-parser-output .navbar-ct-mini{font-size:114%;margin:0 4em}html.skin-theme-clientpref-night .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}@media(prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}}@media print{.mw-parser-output .navbar{display:none!important}}</style><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Soft_tissue_tumors_and_sarcomas" title="Template:Soft tissue tumors and sarcomas"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Soft_tissue_tumors_and_sarcomas" title="Template talk:Soft tissue tumors and sarcomas"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Soft_tissue_tumors_and_sarcomas" title="Special:EditPage/Template:Soft tissue tumors and sarcomas"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Connective/soft_tissue_tumors_and_sarcomas" style="font-size:114%;margin:0 4em"><a href="/wiki/Connective_tissue" title="Connective tissue">Connective</a>/<a href="/wiki/Soft_tissue" title="Soft tissue">soft tissue</a> <a href="/wiki/Neoplasm" title="Neoplasm">tumors</a> and <a href="/wiki/Sarcoma" title="Sarcoma">sarcomas</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%">Not otherwise specified</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Soft-tissue_sarcoma" title="Soft-tissue sarcoma">Soft-tissue sarcoma</a></li> <li><a href="/wiki/Desmoplastic_small-round-cell_tumor" title="Desmoplastic small-round-cell tumor">Desmoplastic small-round-cell tumor</a></li> <li><a href="/wiki/Skin_sarcoma" title="Skin sarcoma">Skin sarcoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Connective_tissue_neoplasm" title="Connective tissue neoplasm">Connective tissue neoplasm</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Fibrous_tissue_neoplasm" title="Fibrous tissue neoplasm">Fibromatous</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Fibroma" title="Fibroma">Fibroma</a>/<a href="/wiki/Fibrosarcoma" title="Fibrosarcoma">fibrosarcoma</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Dermatofibrosarcoma_protuberans" title="Dermatofibrosarcoma protuberans">Dermatofibrosarcoma protuberans</a></li> <li><a href="/wiki/Desmoplastic_fibroma" title="Desmoplastic fibroma">Desmoplastic fibroma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Fibroma" title="Fibroma">Fibroma</a>/<a href="/wiki/Fibromatosis" title="Fibromatosis">fibromatosis</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Aggressive_fibromatosis" title="Aggressive fibromatosis">Aggressive fibromatosis</a></li> <li><a href="/wiki/Aggressive_infantile_fibromatosis" class="mw-redirect" title="Aggressive infantile fibromatosis">Aggressive infantile fibromatosis</a></li> <li><a href="/wiki/Aponeurotic_fibroma" title="Aponeurotic fibroma">Aponeurotic fibroma</a></li> <li><a href="/wiki/Collagenous_fibroma" title="Collagenous fibroma">Collagenous fibroma</a></li> <li><a href="/wiki/Diffuse_infantile_fibromatosis" title="Diffuse infantile fibromatosis">Diffuse infantile fibromatosis</a></li> <li>Familial myxovascular fibromas</li> <li><a href="/wiki/Fibroma_of_tendon_sheath" title="Fibroma of tendon sheath">Fibroma of tendon sheath</a></li> <li><a href="/wiki/Fibromatosis_colli" title="Fibromatosis colli">Fibromatosis colli</a></li> <li><a href="/wiki/Infantile_digital_fibromatosis" title="Infantile digital fibromatosis">Infantile digital fibromatosis</a></li> <li><a href="/wiki/Juvenile_hyaline_fibromatosis" title="Juvenile hyaline fibromatosis">Juvenile hyaline fibromatosis</a></li> <li><a href="/wiki/Plantar_fibromatosis" title="Plantar fibromatosis">Plantar fibromatosis</a></li> <li><a href="/wiki/Pleomorphic_fibroma" title="Pleomorphic fibroma">Pleomorphic fibroma</a></li> <li><a href="/wiki/Oral_submucous_fibrosis" title="Oral submucous fibrosis">Oral submucous fibrosis</a></li> <li><a href="/wiki/Pachydermodactyly" title="Pachydermodactyly">Pachydermodactyly</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Histiocytoma" title="Histiocytoma">Histiocytoma</a>/<a href="/wiki/Histiocytic_sarcoma" title="Histiocytic sarcoma">histiocytic sarcoma</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Benign_fibrous_histiocytoma" class="mw-redirect" title="Benign fibrous histiocytoma">Benign fibrous histiocytoma</a></li> <li><a href="/wiki/Pleomorphic_undifferentiated_sarcoma" class="mw-redirect" title="Pleomorphic undifferentiated sarcoma">Malignant fibrous histiocytoma</a></li> <li><a href="/wiki/Atypical_fibroxanthoma" title="Atypical fibroxanthoma">Atypical fibroxanthoma</a></li> <li><a href="/wiki/Solitary_fibrous_tumor" title="Solitary fibrous tumor">Solitary fibrous tumor</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Myxoma" title="Myxoma">Myxomatous</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Myxoma" title="Myxoma">Myxoma</a>/<a href="/wiki/Myxosarcoma" title="Myxosarcoma">myxosarcoma</a> <ul><li><a href="/wiki/Cutaneous_myxoma" title="Cutaneous myxoma">Cutaneous myxoma</a></li> <li><a href="/wiki/Superficial_acral_fibromyxoma" title="Superficial acral fibromyxoma">Superficial acral fibromyxoma</a></li></ul></li> <li><a href="/wiki/Aggressive_angiomyxoma" title="Aggressive angiomyxoma">Angiomyxoma</a></li> <li><a href="/wiki/Ossifying_fibromyxoid_tumour" title="Ossifying fibromyxoid tumour">Ossifying fibromyxoid tumour</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Fibroepithelial_neoplasms" class="mw-redirect" title="Fibroepithelial neoplasms">Fibroepithelial</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Brenner_tumour" title="Brenner tumour">Brenner tumour</a></li> <li><a href="/wiki/Fibroadenoma" title="Fibroadenoma">Fibroadenoma</a></li> <li><a href="/wiki/Phyllodes_tumor" title="Phyllodes tumor">Phyllodes tumor</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Synovial_joint" title="Synovial joint">Synovial</a>-like</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Synovial_sarcoma" title="Synovial sarcoma">Synovial sarcoma</a></li> <li><a href="/wiki/Clear-cell_sarcoma" class="mw-redirect" title="Clear-cell sarcoma">Clear-cell sarcoma</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Adipose_tissue_neoplasm" title="Adipose tissue neoplasm">Lipomatous</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Lipoma" title="Lipoma">Lipoma</a>/<a href="/wiki/Liposarcoma" title="Liposarcoma">liposarcoma</a> <ul><li><a href="/wiki/Myelolipoma" title="Myelolipoma">Myelolipoma</a></li> <li><a href="/wiki/Myxoid_liposarcoma" title="Myxoid liposarcoma">Myxoid liposarcoma</a></li></ul></li> <li><a href="/wiki/Perivascular_epithelioid_cell_tumour" title="Perivascular epithelioid cell tumour">PEComa</a> <ul><li><a href="/wiki/Angiomyolipoma" title="Angiomyolipoma">Angiomyolipoma</a></li></ul></li></ul> <ul><li><a href="/wiki/Chondroid_lipoma" title="Chondroid lipoma">Chondroid lipoma</a></li> <li><a href="/wiki/Intradermal_spindle_cell_lipoma" class="mw-redirect" title="Intradermal spindle cell lipoma">Intradermal spindle cell lipoma</a></li> <li><a href="/wiki/Pleomorphic_lipoma" title="Pleomorphic lipoma">Pleomorphic lipoma</a></li> <li><a href="/wiki/Lipoblastomatosis" title="Lipoblastomatosis">Lipoblastomatosis</a></li> <li><a href="/wiki/Spindle_cell_lipoma" title="Spindle cell lipoma">Spindle cell lipoma</a></li> <li><a href="/wiki/Hibernoma" title="Hibernoma">Hibernoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Muscle_tissue_neoplasm" class="mw-redirect" title="Muscle tissue neoplasm">Myomatous</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold">General</span></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Myoma" title="Myoma">Myoma</a>/<a href="/wiki/Myosarcoma" title="Myosarcoma">myosarcoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><a href="/wiki/Smooth_muscle_tissue" class="mw-redirect" title="Smooth muscle tissue">Smooth muscle</a></span></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Leiomyoma" title="Leiomyoma">Leiomyoma</a>/<a href="/wiki/Leiomyosarcoma" title="Leiomyosarcoma">leiomyosarcoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><a href="/wiki/Skeletal_muscle" title="Skeletal muscle">Skeletal muscle</a></span></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Rhabdomyoma" title="Rhabdomyoma">Rhabdomyoma</a>/<a class="mw-selflink selflink">rhabdomyosarcoma</a>: <a href="/wiki/Embryonal_rhabdomyosarcoma" title="Embryonal rhabdomyosarcoma">Embryonal rhabdomyosarcoma</a> <ul><li><a href="/wiki/Sarcoma_botryoides" title="Sarcoma botryoides">Sarcoma botryoides</a></li></ul></li> <li><a href="/wiki/Alveolar_rhabdomyosarcoma" title="Alveolar rhabdomyosarcoma">Alveolar rhabdomyosarcoma</a></li></ul> </div></td></tr><tr><td colspan="2" class="navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Leiomyoma" title="Leiomyoma">Leiomyoma</a> <ul><li><a href="/wiki/Cutaneous_leiomyoma" title="Cutaneous leiomyoma">Cutaneous</a></li></ul></li> <li><a href="/wiki/Angioleiomyoma" title="Angioleiomyoma">Angioleiomyoma</a></li> <li><a href="/wiki/Angiolipoleiomyoma" title="Angiolipoleiomyoma">Angiolipoleiomyoma</a></li> <li><a href="/wiki/Genital_leiomyoma" title="Genital leiomyoma">Genital leiomyoma</a></li> <li><a href="/wiki/Leiomyosarcoma" title="Leiomyosarcoma">Leiomyosarcoma</a></li> <li><a href="/wiki/Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome" title="Hereditary leiomyomatosis and renal cell cancer syndrome">Multiple cutaneous and uterine leiomyomatosis syndrome</a></li> <li><a href="/wiki/Multiple_cutaneous_leiomyoma" class="mw-redirect" title="Multiple cutaneous leiomyoma">Multiple cutaneous leiomyoma</a></li> <li><a href="/wiki/Neural_fibrolipoma" title="Neural fibrolipoma">Neural fibrolipoma</a></li> <li><a href="/wiki/Solitary_cutaneous_leiomyoma" class="mw-redirect" title="Solitary cutaneous leiomyoma">Solitary cutaneous leiomyoma</a></li> <li><a href="/wiki/Smooth_muscle_tumor_of_uncertain_malignant_potential" title="Smooth muscle tumor of uncertain malignant potential">STUMP</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Complex_and_mixed_tumor" class="mw-redirect" title="Complex and mixed tumor">Complex mixed and stromal</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Adenomyoma" title="Adenomyoma">Adenomyoma</a></li> <li><a href="/wiki/Pleomorphic_adenoma" title="Pleomorphic adenoma">Pleomorphic adenoma</a></li> <li><a href="/wiki/Mixed_M%C3%BCllerian_tumor" title="Mixed Müllerian tumor">Mixed Müllerian tumor</a></li> <li><a href="/wiki/Mesoblastic_nephroma" title="Mesoblastic nephroma">Mesoblastic nephroma</a></li> <li><a href="/wiki/Wilms%27_tumor" title="Wilms' tumor">Wilms' tumor</a></li> <li><a href="/wiki/Malignant_rhabdoid_tumour" title="Malignant rhabdoid tumour">Malignant rhabdoid tumour</a></li> <li><a href="/wiki/Clear-cell_sarcoma_of_the_kidney" title="Clear-cell sarcoma of the kidney">Clear-cell sarcoma of the kidney</a></li> <li><a href="/wiki/Hepatoblastoma" title="Hepatoblastoma">Hepatoblastoma</a></li> <li><a href="/wiki/Pancreatoblastoma" title="Pancreatoblastoma">Pancreatoblastoma</a></li> <li><a href="/wiki/Carcinosarcoma" title="Carcinosarcoma">Carcinosarcoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Mesothelium" title="Mesothelium">Mesothelial</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Mesothelioma" title="Mesothelioma">Mesothelioma</a></li> <li><a href="/wiki/Adenomatoid_tumor" title="Adenomatoid tumor">Adenomatoid tumor</a></li></ul> </div></td></tr></tbody></table></div>    </div><noscript><img src="https://login.wikimedia.org/wiki/Special:CentralAutoLogin/start?type=1x1" alt="" width="1" height="1" style="border: none; position: absolute;"></noscript> <div class="printfooter" data-nosnippet="">Retrieved from "<a dir="ltr" href="https://en.wikipedia.org/w/index.php?title=Rhabdomyosarcoma&oldid=1238508994">https://en.wikipedia.org/w/index.php?title=Rhabdomyosarcoma&oldid=1238508994</a>"</div></div> <div id="catlinks" class="catlinks" data-mw="interface"><div id="mw-normal-catlinks" class="mw-normal-catlinks"><a href="/wiki/Help:Category" title="Help:Category">Categories</a>: <ul><li><a href="/wiki/Category:Anatomical_pathology" title="Category:Anatomical pathology">Anatomical pathology</a></li><li><a href="/wiki/Category:Connective_and_soft_tissue_neoplasms" 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