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<meta name="robots" content="INDEX,FOLLOW,NOARCHIVE" /><meta name="citation_inbook_title" content="PDQ Cancer Information Summaries [Internet]" /><meta name="citation_title" content="Childhood Cardiac Tumors Treatment (PDQ®)" /><meta name="citation_publisher" content="National Cancer Institute (US)" /><meta name="citation_date" content="2019/10/02" /><meta name="citation_author" content="PDQ Pediatric Treatment Editorial Board" /><meta name="citation_pmid" content="31593384" /><meta name="citation_fulltext_html_url" content="https://www.ncbi.nlm.nih.gov/books/NBK547378/" /><meta name="citation_keywords" content="unusual cancers of childhood" /><meta name="citation_keywords" content="Cardiac tumors" /><meta name="citation_keywords" content="Pediatric cardiac cancer" /><meta name="citation_keywords" content="Pediatric heart cancer" /><link rel="schema.DC" href="http://purl.org/DC/elements/1.0/" /><meta name="DC.Title" content="Childhood Cardiac Tumors Treatment (PDQ®)" /><meta name="DC.Type" content="Text" /><meta name="DC.Publisher" content="National Cancer Institute (US)" /><meta name="DC.Contributor" content="PDQ Pediatric Treatment Editorial Board" /><meta name="DC.Date" content="2019/10/02" /><meta name="DC.Identifier" content="https://www.ncbi.nlm.nih.gov/books/NBK547378/" /><meta name="description" content="This evidence-based, expert-reviewed childhood cardiac tumors summary discusses histology, risk factors, clinical presentation, diagnostic evaluation, and treatment." /><meta name="og:title" content="Childhood Cardiac Tumors Treatment (PDQ®)" /><meta name="og:type" content="book" /><meta name="og:description" content="This evidence-based, expert-reviewed childhood cardiac tumors summary discusses histology, risk factors, clinical presentation, diagnostic evaluation, and treatment." /><meta name="og:url" content="https://www.ncbi.nlm.nih.gov/books/NBK547378/" /><meta name="og:site_name" content="NCBI Bookshelf" /><meta name="og:image" content="https://www.ncbi.nlm.nih.gov/corehtml/pmc/pmcgifs/bookshelf/thumbs/th-pdqcis-lrg.png" /><meta name="twitter:card" content="summary" /><meta name="twitter:site" content="@ncbibooks" /><meta name="bk-non-canon-loc" content="/books/n/pdqcis/CDR0000799419/" /><link rel="canonical" href="https://www.ncbi.nlm.nih.gov/books/NBK547378/" /><link rel="stylesheet" href="/corehtml/pmc/css/figpopup.css" type="text/css" media="screen" /><link rel="stylesheet" href="/corehtml/pmc/css/bookshelf/2.26/css/books.min.css" type="text/css" /><link rel="stylesheet" href="/corehtml/pmc/css/bookshelf/2.26/css/books_print.min.css" type="text/css" media="print" /><style type="text/css">p a.figpopup{display:inline !important} .bk_tt {font-family: monospace} .first-line-outdent .bk_ref {display: inline} .body-content h2, .body-content .h2 {border-bottom: 1px solid #97B0C8} .body-content h2.inline {border-bottom: none} a.page-toc-label , .jig-ncbismoothscroll a {text-decoration:none;border:0 !important} .temp-labeled-list .graphic {display:inline-block !important} .temp-labeled-list img{width:100%}</style><script type="text/javascript" src="/corehtml/pmc/js/jquery.hoverIntent.min.js"> </script><script type="text/javascript" src="/corehtml/pmc/js/common.min.js?_=3.18"> </script><script type="text/javascript" src="/corehtml/pmc/js/large-obj-scrollbars.min.js"> </script><script type="text/javascript">window.name="mainwindow";</script><script type="text/javascript" src="/corehtml/pmc/js/bookshelf/2.26/book-toc.min.js"> </script><script type="text/javascript" src="/corehtml/pmc/js/bookshelf/2.26/books.min.js"> </script><meta name="book-collection" content="NONE" />
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<div class="pre-content"><div><div class="bk_prnt"><p class="small">NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.</p><p>PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. </p></div><div class="iconblock clearfix whole_rhythm no_top_margin bk_noprnt"><a class="img_link icnblk_img" title="Table of Contents Page" href="/books/n/pdqcis/"><img class="source-thumb" src="/corehtml/pmc/pmcgifs/bookshelf/thumbs/th-pdqcis-lrg.png" alt="Cover of PDQ Cancer Information Summaries" height="100px" width="80px" /></a><div class="icnblk_cntnt eight_col"><h2>PDQ Cancer Information Summaries [Internet].</h2><a data-jig="ncbitoggler" href="#__NBK547378_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK547378_dtls__"><div>Bethesda (MD): <a href="http://www.cancer.gov/" ref="pagearea=page-banner&targetsite=external&targetcat=link&targettype=publisher">National Cancer Institute (US)</a>; 2002-.</div></div><div class="half_rhythm"></div><div class="bk_noprnt"><form method="get" action="/books/n/pdqcis/" id="bk_srch"><div class="bk_search"><label for="bk_term" class="offscreen_noflow">Search term</label><input type="text" title="Search this book" id="bk_term" name="term" value="" data-jig="ncbiclearbutton" /> <input type="submit" class="jig-ncbibutton" value="Search this book" submit="false" style="padding: 0.1em 0.4em;" /></div></form></div></div></div></div></div>
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<div class="main-content lit-style" itemscope="itemscope" itemtype="http://schema.org/CreativeWork"><div class="meta-content fm-sec"><h1 id="_NBK547378_"><span class="title" itemprop="name">Childhood Cardiac Tumors Treatment (PDQ®)</span></h1><div class="subtitle whole_rhythm">Health Professional Version</div><p class="contrib-group"><span itemprop="author">PDQ Pediatric Treatment Editorial Board</span>.</p><p class="small">Published online: December 9, 2022.</p><p class="small">Created: <span itemprop="datePublished">October 2, 2019</span>.</p></div><div class="jig-ncbiinpagenav body-content whole_rhythm" data-jigconfig="allHeadingLevels: ['h2'],smoothScroll: false" itemprop="text"><div id="_abs_rndgid_" itemprop="description"><p id="CDR0000799419__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood cardiac tumors. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.</p><p id="CDR0000799419__1557">This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).</p></div><div id="CDR0000799419__1063"><h2 id="_CDR0000799419__1063_">Incidence and Types of Childhood Cardiac Tumors</h2><p id="CDR0000799419__1064">Cardiac tumors are rare in children, with an autopsy frequency of 0.001% to 0.30%.[<a class="bk_pop" href="#CDR0000799419_rl_1063_1">1</a>] In one report, the percentage of cardiac surgeries performed as a result of cardiac tumors was 0.093%.[<a class="bk_pop" href="#CDR0000799419_rl_1063_2">2</a>]</p><p id="CDR0000799419__2340">The distribution of cardiac tumors in the fetal and neonatal period is different from that in older patients, with two-thirds of teratomas occurring during this period of life.[<a class="bk_pop" href="#CDR0000799419_rl_1063_3">3</a>]</p><p id="CDR0000799419__2128">The most common primary tumors of the heart are benign and include the following:[<a class="bk_pop" href="#CDR0000799419_rl_1063_4">4</a>-<a class="bk_pop" href="#CDR0000799419_rl_1063_6">6</a>]</p><ul id="CDR0000799419__1508"><li class="half_rhythm"><div>Rhabdomyoma.</div></li><li class="half_rhythm"><div>Myxoma.</div></li><li class="half_rhythm"><div>Teratoma.</div></li><li class="half_rhythm"><div>Fibroma.</div></li></ul><p id="CDR0000799419__1509"> Other benign tumors include histiocytoid cardiomyopathy tumors, hemangiomas, and neurofibromas (i.e., tumors of the nerves that innervate the muscles).[<a class="bk_pop" href="#CDR0000799419_rl_1063_3">3</a>,<a class="bk_pop" href="#CDR0000799419_rl_1063_4">4</a>,<a class="bk_pop" href="#CDR0000799419_rl_1063_7">7</a>-<a class="bk_pop" href="#CDR0000799419_rl_1063_9">9</a>]</p><p id="CDR0000799419__1066">Primary malignant pediatric heart tumors are rare and include the following:[<a class="bk_pop" href="#CDR0000799419_rl_1063_4">4</a>,<a class="bk_pop" href="#CDR0000799419_rl_1063_10">10</a>-<a class="bk_pop" href="#CDR0000799419_rl_1063_12">12</a>]</p><ul id="CDR0000799419__1510"><li class="half_rhythm"><div> Malignant teratoma.</div></li><li class="half_rhythm"><div>Lymphoma.</div></li><li class="half_rhythm"><div>Various sarcomas, including rhabdomyosarcoma, angiosarcoma, undifferentiated pleomorphic sarcoma, leiomyosarcoma, chondrosarcoma, synovial sarcoma, and infantile fibrosarcoma.</div></li></ul><p id="CDR0000799419__1067">Secondary tumors of the heart include metastatic spread of rhabdomyosarcoma,
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other sarcomas, melanoma, leukemia, thymoma, and carcinomas of various sites.[<a class="bk_pop" href="#CDR0000799419_rl_1063_1">1</a>,<a class="bk_pop" href="#CDR0000799419_rl_1063_4">4</a>] </p><div id="CDR0000799419_rl_1063"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799419_rl_1063_1">Butany J, Nair V, Naseemuddin A, et al.: Cardiac tumours: diagnosis and management. Lancet Oncol 6 (4): 219-28, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15811617" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15811617</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_2">Bielefeld KJ, Moller JH: Cardiac tumors in infants and children: study of 120 operated patients. Pediatr Cardiol 34 (1): 125-8, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/22735896" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22735896</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_3">Isaacs H: Fetal and neonatal cardiac tumors. Pediatr Cardiol 25 (3): 252-73, 2004 May-Jun. [<a href="https://pubmed.ncbi.nlm.nih.gov/15360117" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15360117</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_4">Burke A, Virmani R: Pediatric heart tumors. Cardiovasc Pathol 17 (4): 193-8, 2008 Jul-Aug. [<a href="https://pubmed.ncbi.nlm.nih.gov/18402818" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18402818</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_5">Becker AE: Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians. Pediatr Cardiol 21 (4): 317-23, 2000 Jul-Aug. [<a href="https://pubmed.ncbi.nlm.nih.gov/10865004" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 10865004</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_6">Padalino MA, Vida VL, Boccuzzo G, et al.: Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study. Circulation 126 (1): 22-30, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22626745" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22626745</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_7">Elderkin RA, Radford DJ: Primary cardiac tumours in a paediatric population. J Paediatr Child Health 38 (2): 173-7, 2002. [<a href="https://pubmed.ncbi.nlm.nih.gov/12031001" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12031001</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_8">Uzun O, Wilson DG, Vujanic GM, et al.: Cardiac tumours in children. Orphanet J Rare Dis 2: 11, 2007. [<a href="/pmc/articles/PMC3225855/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3225855</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/17331235" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17331235</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_9">Bruce CJ: Cardiac tumours: diagnosis and management. Heart 97 (2): 151-60, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21163893" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21163893</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_10">Kogon B, Shehata B, Katzenstein H, et al.: Primary congenital infantile fibrosarcoma of the heart: the first confirmed case. Ann Thorac Surg 91 (4): 1276-80, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21440163" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21440163</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_11">Wang JG, Li NN: Primary cardiac synovial sarcoma. Ann Thorac Surg 95 (6): 2202-9, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23647858" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23647858</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1063_12">Ostrowski S, Marcinkiewicz A, Kośmider A, et al.: Sarcomas of the heart as a difficult interdisciplinary problem. Arch Med Sci 10 (1): 135-48, 2014. [<a href="/pmc/articles/PMC3953983/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3953983</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/24701226" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24701226</span></a>]</div></li></ol></div></div><div id="CDR0000799419__1068"><h2 id="_CDR0000799419__1068_">Risk Factors</h2><p id="CDR0000799419__1069">Multiple cardiac tumors noted in the fetal or neonatal period are highly associated with a diagnosis of tuberous sclerosis.[<a class="bk_pop" href="#CDR0000799419_rl_1068_1">1</a>,<a class="bk_pop" href="#CDR0000799419_rl_1068_2">2</a>] </p><p id="CDR0000799419__2341">A retrospective review of 94 patients with cardiac tumors detected by prenatal or neonatal echocardiography showed that 68% of the patients exhibited features of tuberous sclerosis.[<a class="bk_pop" href="#CDR0000799419_rl_1068_3">3</a>] In another study, 79% of patients (15 of 19) with rhabdomyomas discovered prenatally had tuberous sclerosis, while 96% of those diagnosed postnatally had tuberous sclerosis. </p><p id="CDR0000799419__1065">Carney complex is a risk factor for developing myxomas. Carney complex is a rare syndrome characterized by lentigines, cardiac myxomas or other myxoid fibromas, and endocrine abnormalities.[<a class="bk_pop" href="#CDR0000799419_rl_1068_4">4</a>-<a class="bk_pop" href="#CDR0000799419_rl_1068_6">6</a>]
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A mutation of the <i>PRKAR1A</i> gene is noted in more than 90% of Carney complex cases.[<a class="bk_pop" href="#CDR0000799419_rl_1068_4">4</a>,<a class="bk_pop" href="#CDR0000799419_rl_1068_7">7</a>]</p><div id="CDR0000799419_rl_1068"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799419_rl_1068_1">Isaacs H: Fetal and neonatal cardiac tumors. Pediatr Cardiol 25 (3): 252-73, 2004 May-Jun. [<a href="https://pubmed.ncbi.nlm.nih.gov/15360117" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15360117</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1068_2">Kocabaş A, Ekici F, Cetin Iİ, et al.: Cardiac rhabdomyomas associated with tuberous sclerosis complex in 11 children: presentation to outcome. Pediatr Hematol Oncol 30 (2): 71-9, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23151153" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23151153</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1068_3">Tworetzky W, McElhinney DB, Margossian R, et al.: Association between cardiac tumors and tuberous sclerosis in the fetus and neonate. Am J Cardiol 92 (4): 487-9, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12914889" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12914889</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1068_4">Boikos SA, Stratakis CA: Carney complex: the first 20 years. Curr Opin Oncol 19 (1): 24-9, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17133108" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17133108</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1068_5">Carney JA, Young WF: Primary pigmented nodular adrenocortical disease and its associated conditions. Endocrinologist 2: 6-21, 1992.</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1068_6">Stratakis CA, Kirschner LS, Carney JA: Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab 86 (9): 4041-6, 2001. [<a href="https://pubmed.ncbi.nlm.nih.gov/11549623" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11549623</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1068_7">Boikos SA, Stratakis CA: Carney complex: pathology and molecular genetics. Neuroendocrinology 83 (3-4): 189-99, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/17047382" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17047382</span></a>]</div></li></ol></div></div><div id="CDR0000799419__1070"><h2 id="_CDR0000799419__1070_">Clinical Presentation and Diagnostic Evaluation</h2><p id="CDR0000799419__1071">Patients may be asymptomatic and present with sudden death,[<a class="bk_pop" href="#CDR0000799419_rl_1070_1">1</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>] but about two-thirds of patients have symptoms that may include the following:</p><ul id="CDR0000799419__1270"><li class="half_rhythm"><div>Abnormalities of heart rhythm.</div></li><li class="half_rhythm"><div>Enlargement of
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the heart.</div></li><li class="half_rhythm"><div>Fluid in the pericardial sac.</div></li><li class="half_rhythm"><div>Congestive heart failure.</div></li><li class="half_rhythm"><div>Syncope.</div></li><li class="half_rhythm"><div>Stroke.</div></li><li class="half_rhythm"><div>Respiratory distress.[<a class="bk_pop" href="#CDR0000799419_rl_1070_2">2</a>]
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</div></li></ul><p id="CDR0000799419__1072">Cardiac magnetic resonance imaging using specific sequences can lead to an accurate diagnosis in most patients with cardiac tumors.[<a class="bk_pop" href="#CDR0000799419_rl_1070_3">3</a>,<a class="bk_pop" href="#CDR0000799419_rl_1070_4">4</a>] However, a histological diagnosis should remain the standard in cases where the diagnosis cannot be established by noninvasive methods or if the possibility of malignancy has been considered.</p><div id="CDR0000799419_rl_1070"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799419_rl_1070_1">Neri M, Di Donato S, Maglietta R, et al.: Sudden death as presenting symptom caused by cardiac primary multicentric left ventricle rhabdomyoma, in an 11-month-old baby. An immunohistochemical study. Diagn Pathol 7: 169, 2012. [<a href="/pmc/articles/PMC3520811/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3520811</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/23206573" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23206573</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1070_2">Padalino MA, Vida VL, Boccuzzo G, et al.: Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study. Circulation 126 (1): 22-30, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22626745" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22626745</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1070_3">Beroukhim RS, Ghelani S, Ashwath R, et al.: Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study. JACC Cardiovasc Imaging 15 (8): 1391-1405, 2022. [<a href="https://pubmed.ncbi.nlm.nih.gov/34419404" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 34419404</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1070_4">Beroukhim RS, Prakash A, Buechel ER, et al.: Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience. J Am Coll Cardiol 58 (10): 1044-54, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21867841" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21867841</span></a>]</div></li></ol></div></div><div id="CDR0000799419__1073"><h2 id="_CDR0000799419__1073_">Treatment of Childhood Cardiac Tumors</h2><p id="CDR0000799419__1074">Successful treatment may require surgery, debulking for progressive symptoms, cardiac transplant, and
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chemotherapy that is appropriate for the type of cancer that is present.[<a class="bk_pop" href="#CDR0000799419_rl_1073_1">1</a>-<a class="bk_pop" href="#CDR0000799419_rl_1073_3">3</a>]; [<a class="bk_pop" href="#CDR0000799419_rl_1073_4">4</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>] In one series, 95% of patients were free from cardiac tumor recurrence at 10 years.[<a class="bk_pop" href="#CDR0000799419_rl_1073_5">5</a>]</p><p id="CDR0000799419__2129">Treatment options for childhood cardiac tumors, according to tumor type or resectability, are as follows:</p><div id="CDR0000799419__2342"><h3>Rhabdomyoma</h3><p id="CDR0000799419__2343">Most rhabdomyomas, whether diagnosed prenatally or postnatally, will spontaneously regress.[<a class="bk_pop" href="#CDR0000799419_rl_1073_6">6</a>] However, although some lesions can regress spontaneously, certain practitioners may recommend prophylactic resection to prevent mass-related complications.[<a class="bk_pop" href="#CDR0000799419_rl_1073_5">5</a>,<a class="bk_pop" href="#CDR0000799419_rl_1073_7">7</a>,<a class="bk_pop" href="#CDR0000799419_rl_1073_8">8</a>]; [<a class="bk_pop" href="#CDR0000799419_rl_1073_9">9</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810037/" class="def">Level of evidence C2</a>] </p><p id="CDR0000799419__2351">Treatment with the mammalian target of rapamycin (mTOR) inhibitors everolimus or sirolimus has been reported to be associated with a decrease in the size of rhabdomyomas in patients with tuberous sclerosis.[<a class="bk_pop" href="#CDR0000799419_rl_1073_8">8</a>,<a class="bk_pop" href="#CDR0000799419_rl_1073_10">10</a>-<a class="bk_pop" href="#CDR0000799419_rl_1073_13">13</a>]</p></div><div id="CDR0000799419__2344"><h3>Sarcoma</h3><p id="CDR0000799419__2345">Patients with cardiac sarcomas have a poor outcome and can be treated with multimodal therapy. The use of preoperative chemotherapy and/or radiation therapy may be of value in reducing tumor volume before surgery. </p></div><div id="CDR0000799419__2346"><h3>Other Tumor Types</h3><p id="CDR0000799419__2347">Complete surgical excision of other lesions offers the best chance for cure. Postoperative complications are seen in about one-third of patients, and postoperative mortality rates are less than 10%.[<a class="bk_pop" href="#CDR0000799419_rl_1073_5">5</a>,<a class="bk_pop" href="#CDR0000799419_rl_1073_7">7</a>,<a class="bk_pop" href="#CDR0000799419_rl_1073_14">14</a>] </p></div><div id="CDR0000799419__2348"><h3>Unresectable Tumor</h3><p id="CDR0000799419__2349">Radiation therapy is a rare treatment option for patients with unresectable disease. Radiation therapy is used to prevent progression because it is unlikely to produce full disease resolution.[<a class="bk_pop" href="#CDR0000799419_rl_1073_15">15</a>-<a class="bk_pop" href="#CDR0000799419_rl_1073_18">18</a>]</p></div><div id="CDR0000799419_rl_1073"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799419_rl_1073_1">Michler RE, Goldstein DJ: Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol 24 (5): 534-9, 1997. [<a href="https://pubmed.ncbi.nlm.nih.gov/9344319" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9344319</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_2">Stiller B, Hetzer R, Meyer R, et al.: Primary cardiac tumours: when is surgery necessary? Eur J Cardiothorac Surg 20 (5): 1002-6, 2001. [<a href="https://pubmed.ncbi.nlm.nih.gov/11675188" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11675188</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_3">Günther T, Schreiber C, Noebauer C, et al.: Treatment strategies for pediatric patients with primary cardiac and pericardial tumors: a 30-year review. Pediatr Cardiol 29 (6): 1071-6, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18600370" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18600370</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_4">Wu KH, Mo XM, Liu YL: Clinical analysis and surgical results of cardiac myxoma in pediatric patients. J Surg Oncol 99 (1): 48-50, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/18980220" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18980220</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_5">Padalino MA, Vida VL, Boccuzzo G, et al.: Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study. Circulation 126 (1): 22-30, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22626745" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22626745</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_6">Bader RS, Chitayat D, Kelly E, et al.: Fetal rhabdomyoma: prenatal diagnosis, clinical outcome, and incidence of associated tuberous sclerosis complex. J Pediatr 143 (5): 620-4, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/14615733" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 14615733</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_7">Bielefeld KJ, Moller JH: Cardiac tumors in infants and children: study of 120 operated patients. Pediatr Cardiol 34 (1): 125-8, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/22735896" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22735896</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_8">Kocabaş A, Ekici F, Cetin Iİ, et al.: Cardiac rhabdomyomas associated with tuberous sclerosis complex in 11 children: presentation to outcome. Pediatr Hematol Oncol 30 (2): 71-9, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23151153" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23151153</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_9">Kutluk T, Demir HA, Büyükpamukçu M, et al.: Cardiac rhabdomyomas in childhood: six cases from a single institution. Turk J Pediatr 55 (1): 69-73, 2013 Jan-Feb. [<a href="https://pubmed.ncbi.nlm.nih.gov/23692835" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23692835</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_10">Choudhry S, Nguyen HH, Anwar S: Rapid resolution of cardiac rhabdomyomas following everolimus therapy. BMJ Case Rep 2015: , 2015. [<a href="/pmc/articles/PMC4680252/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4680252</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/26661560" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26661560</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_11">Barnes BT, Procaccini D, Crino J, et al.: Maternal Sirolimus Therapy for Fetal Cardiac Rhabdomyomas. N Engl J Med 378 (19): 1844-1845, 2018. [<a href="/pmc/articles/PMC6201692/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6201692</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/29742370" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29742370</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_12">Nir-David Y, Brosilow S, Khoury A: Rapid response of a cardiac rhabdomyoma causing severe right ventricular outflow obstruction to Sirolimus in an infant with negative genetics for Tuberous sclerosis. Cardiol Young 31 (2): 312-314, 2021. [<a href="https://pubmed.ncbi.nlm.nih.gov/33148352" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33148352</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_13">Chen XQ, Wang YY, Zhang MN, et al.: Sirolimus Can Increase the Disappearance Rate of Cardiac Rhabdomyomas Associated with Tuberous Sclerosis: A Prospective Cohort and Self-Controlled Case Series Study. J Pediatr 233: 150-155.e4, 2021. [<a href="https://pubmed.ncbi.nlm.nih.gov/33631166" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33631166</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_14">Beroukhim RS, Geva T, Del Nido P, et al.: Risk Factors for Left Ventricular Dysfunction Following Surgical Management of Cardiac Fibroma. Circ Cardiovasc Imaging 14 (2): e011748, 2021. [<a href="https://pubmed.ncbi.nlm.nih.gov/33517672" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33517672</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_15">Movsas B, Teruya-Feldstein J, Smith J, et al.: Primary cardiac sarcoma: a novel treatment approach. Chest 114 (2): 648-52, 1998. [<a href="https://pubmed.ncbi.nlm.nih.gov/9726764" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9726764</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_16">Simpson L, Kumar SK, Okuno SH, et al.: Malignant primary cardiac tumors: review of a single institution experience. Cancer 112 (11): 2440-6, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18428209" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18428209</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_17">Mery GM, Reardon MJ, Haas J, et al.: A combined modality approach to recurrent cardiac sarcoma resulting in a prolonged remission: a case report. Chest 123 (5): 1766-8, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12740300" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12740300</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1073_18">Zerkowski HR, Hofmann HS, Gybels I, et al.: Primary sarcoma of pulmonary artery and valve: multimodality treatment by chemotherapy and homograft replacement. J Thorac Cardiovasc Surg 112 (4): 1122-4, 1996. [<a href="https://pubmed.ncbi.nlm.nih.gov/8873746" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 8873746</span></a>]</div></li></ol></div></div><div id="CDR0000799419__1925"><h2 id="_CDR0000799419__1925_">Treatment Options Under Clinical Evaluation for Childhood Cardiac Tumors</h2><p id="CDR0000799419__1926">Information about National Cancer Institute (NCI)–supported clinical trials can be found on the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/search" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>. For information about clinical trials sponsored by other organizations, see the <a href="https://clinicaltrials.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a>.</p><p id="CDR0000799419__1927">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000799419__1928"><li class="half_rhythm"><div class="half_rhythm"><b><a href="https://www.cancer.gov/clinicaltrials/NCT03155620" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">APEC1621 (NCT03155620)</a></b> (Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients with Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders)<b>:</b> NCI-COG Pediatric Molecular Analysis for Therapeutic Choice (MATCH), referred to as Pediatric MATCH, will match targeted agents with specific molecular changes identified in a patient's tumor (refractory or recurrent). Children and adolescents aged 1 to 21 years are eligible for the trial.</div><div class="half_rhythm">Patients with tumors that have molecular variants addressed by open treatment arms in the trial may be enrolled in treatment on Pediatric MATCH. Additional information can be obtained on the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/nci-supported/pediatric-match" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a> and <a href="https://clinicaltrials.gov/ct2/show/NCT03155620" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a>.
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</div></li></ul></div><div id="CDR0000799419__1864"><h2 id="_CDR0000799419__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000799419__1865">Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[<a class="bk_pop" href="#CDR0000799419_rl_1864_1">1</a>] Referral to medical centers with multidisciplinary teams of cancer specialists experienced in treating cancers that occur in childhood and adolescence should be considered. This multidisciplinary team approach incorporates the skills
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of the following health care professionals and others to ensure that children receive treatment, supportive care, and rehabilitation
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that will achieve optimal survival and quality of life:</p><ul id="CDR0000799419__1866"><li class="half_rhythm"><div>Primary care physicians.</div></li><li class="half_rhythm"><div>Pediatric surgeons.</div></li><li class="half_rhythm"><div>Radiation
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oncologists.</div></li><li class="half_rhythm"><div>Pediatric medical oncologists/hematologists.</div></li><li class="half_rhythm"><div> Rehabilitation
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specialists.</div></li><li class="half_rhythm"><div>Pediatric nurse specialists.</div></li><li class="half_rhythm"><div>Social workers.</div></li><li class="half_rhythm"><div>Child-life professionals.</div></li><li class="half_rhythm"><div>Psychologists.</div></li></ul><p id="CDR0000799419__1867"> For information about supportive care for children and adolescents with cancer, see the summaries on <a href="https://www.cancer.gov/publications/pdq/information-summaries/supportive-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Supportive and Palliative Care</a>.</p><p id="CDR0000799419__1868">The American Academy of Pediatrics has outlined guidelines for
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pediatric cancer centers and their role in the treatment of pediatric patients
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with cancer.[<a class="bk_pop" href="#CDR0000799419_rl_1864_2">2</a>] At
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these pediatric cancer centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity
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to participate is offered to most patients and their families. Clinical
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trials for children and adolescents diagnosed with cancer are generally
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designed to compare potentially better therapy with current standard therapy. Most of the progress made in identifying curative
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therapy for childhood cancers has been achieved through clinical trials.
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Information about ongoing clinical trials is available from the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>.</p><p id="CDR0000799419__1869">Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[<a class="bk_pop" href="#CDR0000799419_rl_1864_3">3</a>-<a class="bk_pop" href="#CDR0000799419_rl_1864_5">5</a>] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. For information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors, see <a href="/books/n/pdqcis/CDR0000343584/">Late Effects of Treatment for Childhood Cancer</a>.</p><p id="CDR0000799419__1870">Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years.[<a class="bk_pop" href="#CDR0000799419_rl_1864_6">6</a>] The U.S. <a href="https://www.congress.gov/107/plaws/publ280/PLAW-107publ280.pdf" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Rare Diseases Act of 2002</a> defines a rare disease as one that affects populations smaller than 200,000 people. Therefore, all pediatric cancers are considered rare.</p><p id="CDR0000799419__1888">The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[<a class="bk_pop" href="#CDR0000799419_rl_1864_7">7</a>,<a class="bk_pop" href="#CDR0000799419_rl_1864_8">8</a>] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000799419__1871"><li class="half_rhythm"><div class="half_rhythm">A consensus effort between the European Union Joint Action on Rare Cancers and the European Cooperative Study Group for Rare Pediatric Cancers estimated that 11% of all cancers in patients younger than 20 years could be categorized as very rare. This consensus group defined very rare cancers as those with annual incidences of fewer than 2 cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than 2 cases per 1 million people were also included in the very rare group because there is a lack of knowledge and expertise in the management of these tumors.[<a class="bk_pop" href="#CDR0000799419_rl_1864_9">9</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">The Children's Oncology Group (COG) defines rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancers, melanomas and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinomas, nasopharyngeal carcinomas, and most adult-type carcinomas such as breast cancers, colorectal cancers, etc.).[<a class="bk_pop" href="#CDR0000799419_rl_1864_10">10</a>] These diagnoses account for about 5% of the cancers diagnosed in children aged 0 to 14 years and about 27% of the cancers diagnosed in adolescents aged 15 to 19 years.[<a class="bk_pop" href="#CDR0000799419_rl_1864_4">4</a>]</div><div class="half_rhythm"> Most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers in children aged 0 to 14 years and 9.3% of the cancers in adolescents aged 15 to 19 years.</div></li></ul><p id="CDR0000799419__1872">These rare cancers are extremely challenging to study because of the low number of patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the lack of clinical trials for adolescents with rare cancers.</p><div id="CDR0000799419_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799419_rl_1864_1">Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010. [<a href="/pmc/articles/PMC2881732/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2881732</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20404250" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20404250</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1864_2">American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. <a href="https://pediatrics.aappublications.org/content/134/2/410" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Also available online</a>. Last accessed December 15, 2023.</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1864_3">Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014. [<a href="/pmc/articles/PMC4136455/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4136455</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/24853691" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24853691</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1864_4">National Cancer Institute: NCCR*Explorer: An interactive website for NCCR cancer statistics. Bethesda, MD: National Cancer Institute. <a href="https://NCCRExplorer.ccdi.cancer.gov/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online</a>. Last accessed December 15, 2023.</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1864_5">Surveillance Research Program, National Cancer Institute: SEER*Explorer: An interactive website for SEER cancer statistics. Bethesda, MD: National Cancer Institute. <a href="https://seer.cancer.gov/statistics-network/explorer/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online</a>. Last accessed August 18, 2023.</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1864_6">Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr. [<a href="https://pubmed.ncbi.nlm.nih.gov/24488779" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24488779</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1864_7">Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28687376" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28687376</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1864_8">DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28542893" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28542893</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1864_9">Ferrari A, Brecht IB, Gatta G, et al.: Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 110: 120-126, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/30785015" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30785015</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799419_rl_1864_10">Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010. [<a href="/pmc/articles/PMC3020699/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3020699</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20956621" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20956621</span></a>]</div></li></ol></div></div><div id="CDR0000799419__2130"><h2 id="_CDR0000799419__2130_">Latest Updates to This Summary (12/09/2022)</h2><p id="CDR0000799419__2131">The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.</p><p id="CDR0000799419__2460">
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<b>
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<a href="#CDR0000799419__1864">Special Considerations for the Treatment of Children With Cancer</a>
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</b>
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</p><p id="CDR0000799419__2457">Revised <a href="#CDR0000799419__1869">text</a> to state that between 1975 and 2020, childhood cancer mortality decreased by more than 50% (cited National Cancer Institute as reference 4 and Surveillance Research Program, National Cancer Institute as reference 5).</p><p id="CDR0000799419__2458">Revised <a href="/books/NBK547378.5/#CDR0000799419__1871">text</a> to state that rare pediatric cancers account for about 5% of the cancers diagnosed in children aged 0 to 14 years and about 27% of the cancers diagnosed in adolescents aged 15 to 19 years. Also revised text to state that most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers in children aged 0 to 14 years and 9.3% of cancers in adolescents aged 15 to 19 years.</p><p id="CDR0000799419__disclaimerHP_3">This summary is written and maintained by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric Treatment Editorial Board</a>, which is
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editorially independent of NCI. The summary reflects an independent review of
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the literature and does not represent a policy statement of NCI or NIH. More
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information about summary policies and the role of the PDQ Editorial Boards in
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maintaining the PDQ summaries can be found on the <a href="#CDR0000799419__AboutThis_1">About This PDQ Summary</a> and <a href="https://www.cancer.gov/publications/pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ® Cancer Information for Health Professionals</a> pages.
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</p></div><div id="CDR0000799419__AboutThis_1"><h2 id="_CDR0000799419__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000799419__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000799419__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood cardiac tumors. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.</p></div><div id="CDR0000799419__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000799419__AboutThis_5">This summary is reviewed regularly and updated as necessary by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric Treatment Editorial Board</a>, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).</p><p id="CDR0000799419__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000799419__AboutThis_6"><li class="half_rhythm"><div>be discussed at a meeting,</div></li><li class="half_rhythm"><div>be cited with text, or</div></li><li class="half_rhythm"><div>replace or update an existing article that is already cited.</div></li></ul><p id="CDR0000799419__AboutThis_7">Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.</p><p>The lead reviewers for Childhood Cardiac Tumors Treatment are:</p><ul><li class="half_rhythm"><div>Denise Adams, MD (Children's Hospital Boston)</div></li><li class="half_rhythm"><div>Karen J. Marcus, MD, FACR (Dana-Farber Cancer Institute/Boston Children's Hospital)</div></li><li class="half_rhythm"><div>William H. Meyer, MD</div></li><li class="half_rhythm"><div>Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)</div></li><li class="half_rhythm"><div>Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)</div></li><li class="half_rhythm"><div>Alberto S. Pappo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)</div></li><li class="half_rhythm"><div>Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>Stephen J. Shochat, MD (St. Jude Children's Research Hospital)</div></li></ul><p id="CDR0000799419__AboutThis_9">Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.</p></div><div id="CDR0000799419__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000799419__AboutThis_11">Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a <a href="/books/n/pdqcis/CDR0000062796/">formal evidence ranking system</a> in developing its level-of-evidence designations.</p></div><div id="CDR0000799419__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000799419__AboutThis_13">PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”</p><p id="CDR0000799419__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000799419__AboutThis_15">PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Cardiac Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/cardiac/hp-child-cardiac-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/cardiac/hp-child-cardiac-treatment-pdq</a>. Accessed <MM/DD/YYYY>. </p><p id="CDR0000799419__AboutThis_16">Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in <a href="https://visualsonline.cancer.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Visuals Online</a>, a collection of over 2,000 scientific images.
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</p></div><div id="CDR0000799419__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000799419__AboutThis_18">Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the <a href="https://www.cancer.gov/about-cancer/managing-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Managing Cancer Care</a> page.</p></div><div id="CDR0000799419__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000799419__AboutThis_21">More information about contacting us or receiving help with the Cancer.gov website can be found on our <a href="https://www.cancer.gov/contact" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Contact Us for Help</a> page. Questions can also be submitted to Cancer.gov through the website’s <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>.</p></div></div></div></div>
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<div xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance"></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Views</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="PDF_download" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="/books/NBK547378.5/?report=reader">PubReader</a></li><li><a href="/books/NBK547378.5/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK547378" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK547378" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Pediatric Treatment Editorial Board. Childhood Cardiac Tumors Treatment (PDQ®): Health Professional Version. 2022 Dec 9. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. <span class="bk_cite_avail"></span></div></div></li><li><a href="#" class="toggle-glossary-link" title="Enable/disable links to the glossary">Disable Glossary Links</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Version History</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter shutter_closed" title="Show/hide content" remembercollapsed="true" pgsec_name="version_history" id="Shutter"></a></div><div class="portlet_content" style="display: none;"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><span class="bk_col_itm"><a href="/books/NBK547378.8/">NBK547378.8</a></span> September 12, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK547378.7/">NBK547378.7</a></span> April 26, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK547378.6/">NBK547378.6</a></span> January 3, 2024</li><li><span class="bk_col_itm">NBK547378.5</span> December 9, 2022 (Displayed Version)</li><li><span class="bk_col_itm"><a href="/books/NBK547378.4/">NBK547378.4</a></span> November 3, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK547378.3/">NBK547378.3</a></span> May 25, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK547378.2/">NBK547378.2</a></span> October 5, 2021</li><li><span class="bk_col_itm"><a href="/books/NBK547378.1/">NBK547378.1</a></span> October 2, 2019</li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>In this Page</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="page-toc" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="#CDR0000799419__1063" ref="log$=inpage&link_id=inpage">Incidence and Types of Childhood Cardiac Tumors</a></li><li><a href="#CDR0000799419__1068" ref="log$=inpage&link_id=inpage">Risk Factors</a></li><li><a href="#CDR0000799419__1070" ref="log$=inpage&link_id=inpage">Clinical Presentation and Diagnostic Evaluation</a></li><li><a href="#CDR0000799419__1073" ref="log$=inpage&link_id=inpage">Treatment of Childhood Cardiac Tumors</a></li><li><a href="#CDR0000799419__1925" ref="log$=inpage&link_id=inpage">Treatment Options Under Clinical Evaluation for Childhood Cardiac Tumors</a></li><li><a href="#CDR0000799419__1864" ref="log$=inpage&link_id=inpage">Special Considerations for the Treatment of Children With Cancer</a></li><li><a href="#CDR0000799419__2130" ref="log$=inpage&link_id=inpage">Latest Updates to This Summary (12/09/2022)</a></li><li><a href="#CDR0000799419__AboutThis_1" ref="log$=inpage&link_id=inpage">About This PDQ Summary</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related publications</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="document-links" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="/books/NBK547382/">Patient Version</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related information</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="discovery_db_links" id="Shutter"></a></div><div class="portlet_content"><ul><li class="brieflinkpopper"><a class="brieflinkpopperctrl" 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xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Laryngeal Tumors Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Laryngeal Tumors Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389243" 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