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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 Nasopharyngeal Cancer Treatment (PDQ®)" /><meta name="citation_publisher" content="National Cancer Institute (US)" /><meta name="citation_date" content="2018/01/05" /><meta name="citation_author" content="PDQ Pediatric Treatment Editorial Board" /><meta name="citation_pmid" content="29320137" /><meta name="citation_fulltext_html_url" content="https://www.ncbi.nlm.nih.gov/books/NBK475701/" /><meta name="citation_keywords" content="nasopharyngeal cancer" /><meta name="citation_keywords" content="Nasopharyngeal cancer" /><link rel="schema.DC" href="http://purl.org/DC/elements/1.0/" /><meta name="DC.Title" content="Childhood Nasopharyngeal Cancer 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="2018/01/05" /><meta name="DC.Identifier" content="https://www.ncbi.nlm.nih.gov/books/NBK475701/" /><meta name="description" content="Treatment options for children with nasopharyngeal cancer include combined-modality therapy with chemotherapy and radiation. Surgery has a limited role because the disease is usually considered unresectable due to extensive local spread. Get detailed treatment information in this clinician summary." /><meta name="og:title" content="Childhood Nasopharyngeal Cancer Treatment (PDQ®)" /><meta name="og:type" content="book" /><meta name="og:description" content="Treatment options for children with nasopharyngeal cancer include combined-modality therapy with chemotherapy and radiation. Surgery has a limited role because the disease is usually considered unresectable due to extensive local spread. Get detailed treatment information in this clinician summary." /><meta name="og:url" content="https://www.ncbi.nlm.nih.gov/books/NBK475701/" /><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/CDR0000784522/" /><link rel="canonical" href="https://www.ncbi.nlm.nih.gov/books/NBK475701/" /><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="#__NBK475701_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK475701_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="_NBK475701_"><span class="title" itemprop="name">Childhood Nasopharyngeal Cancer Treatment (PDQ®)</span></h1><div class="subtitle whole_rhythm">Health Professional Version</div><p class="contrib-group"><span itemprop="author">PDQ Pediatric Treatment Editorial Board</span>.</p><p class="small">Published online: September 21, 2018.</p><p class="small">Created: <span itemprop="datePublished">January 5, 2018</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="CDR0000784522__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood nasopharyngeal cancer. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.</p><p id="CDR0000784522__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="CDR0000784522__905"><h2 id="_CDR0000784522__905_">Incidence</h2><p id="CDR0000784522__906">Nasopharyngeal carcinoma arises in the lining of the nasal cavity and pharynx, and it accounts for about one-third of all cancers of the upper airways in children.[<a class="bk_pop" href="#CDR0000784522_rl_905_1">1</a>,<a class="bk_pop" href="#CDR0000784522_rl_905_2">2</a>]</p><p id="CDR0000784522__1880">
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Nasopharyngeal carcinoma is very uncommon in children younger than 10 years but increases in incidence to 0.8 cases per 1 million per year in children aged 10 to 14 years and 1.3 cases per million per year in children aged 15 to 19 years.[<a class="bk_pop" href="#CDR0000784522_rl_905_3">3</a>-<a class="bk_pop" href="#CDR0000784522_rl_905_5">5</a>]</p><p id="CDR0000784522__1234">The incidence of nasopharyngeal carcinoma is characterized by racial and geographic variations, with an endemic distribution among well-defined ethnic groups, such as inhabitants of some areas in North Africa and the Mediterranean basin, and, particularly, Southeast Asia. In the United States, the incidence of nasopharyngeal carcinoma is higher in black children and adolescents younger than 20 years.[<a class="bk_pop" href="#CDR0000784522_rl_905_4">4</a>,<a class="bk_pop" href="#CDR0000784522_rl_905_5">5</a>]</p><div id="CDR0000784522_rl_905"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_rl_905_1">Ayan I, Kaytan E, Ayan N: Childhood nasopharyngeal carcinoma: from biology to treatment. Lancet Oncol 4 (1): 13-21, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12517535" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12517535</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_905_2">Yan Z, Xia L, Huang Y, et al.: Nasopharyngeal carcinoma in children and adolescents in an endemic area: a report of 185 cases. Int J Pediatr Otorhinolaryngol 77 (9): 1454-60, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23830224" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23830224</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_905_3">Horner MJ, Ries LA, Krapcho M, et al.: SEER Cancer Statistics Review, 1975-2006. Bethesda, Md: National Cancer Institute, 2009. <a href="https://seer.cancer.gov/archive/csr/1975_2006/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Also available online</a>. Last accessed January 31, 2019.</div></li><li><div class="bk_ref" id="CDR0000784522_rl_905_4">Sultan I, Casanova M, Ferrari A, et al.: Differential features of nasopharyngeal carcinoma in children and adults: a SEER study. Pediatr Blood Cancer 55 (2): 279-84, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20582982" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20582982</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_905_5">Richards MK, Dahl JP, Gow K, et al.: Factors Associated With Mortality in Pediatric vs Adult Nasopharyngeal Carcinoma. JAMA Otolaryngol Head Neck Surg 142 (3): 217-22, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26769566" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26769566</span></a>]</div></li></ol></div></div><div id="CDR0000784522__907"><h2 id="_CDR0000784522__907_">Risk Factors</h2><p id="CDR0000784522__908">Nasopharyngeal carcinoma is strongly associated with Epstein-Barr virus (EBV) infection. In addition to the serological evidence of infection in more than 98% of patients, EBV DNA is present as a monoclonal episome in the nasopharyngeal carcinoma cells, and tumor cells can have EBV antigens on their cell surface.[<a class="bk_pop" href="#CDR0000784522_rl_907_1">1</a>] The circulating levels of EBV DNA and serologic documentation of EBV infection may aid in the diagnosis.[<a class="bk_pop" href="#CDR0000784522_rl_907_2">2</a>] Specific HLA subtypes, such as the HLA A2Bsin2 haplotype, are associated with a higher risk of nasopharyngeal carcinoma.[<a class="bk_pop" href="#CDR0000784522_rl_907_3">3</a>]</p><div id="CDR0000784522_rl_907"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_rl_907_1">Dawson CW, Port RJ, Young LS: The role of the EBV-encoded latent membrane proteins LMP1 and LMP2 in the pathogenesis of nasopharyngeal carcinoma (NPC). Semin Cancer Biol 22 (2): 144-53, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22249143" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22249143</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_907_2">Lo YM, Chan LY, Lo KW, et al.: Quantitative analysis of cell-free Epstein-Barr virus DNA in plasma of patients with nasopharyngeal carcinoma. Cancer Res 59 (6): 1188-91, 1999. [<a href="https://pubmed.ncbi.nlm.nih.gov/10096545" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 10096545</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_907_3">Ayan I, Kaytan E, Ayan N: Childhood nasopharyngeal carcinoma: from biology to treatment. Lancet Oncol 4 (1): 13-21, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12517535" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12517535</span></a>]</div></li></ol></div></div><div id="CDR0000784522__909"><h2 id="_CDR0000784522__909_">Histology</h2><p id="CDR0000784522__910">Three histologic subtypes of nasopharyngeal carcinoma are recognized by the World
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Health Organization (WHO): </p><ul id="CDR0000784522__1235"><li class="half_rhythm"><div>Type I—keratinizing squamous cell carcinoma.</div></li><li class="half_rhythm"><div>Type II—nonkeratinizing squamous cell carcinoma. Type II is distinguished by the presence of lymphoid infiltration as type IIa or IIb.</div></li><li class="half_rhythm"><div>Type III—undifferentiated carcinoma. Type III is distinguished by the presence of lymphoid infiltration as type IIIa or IIIb.</div></li></ul><p id="CDR0000784522__1236"> Children with nasopharyngeal carcinoma are more likely to have WHO type II or type III disease.[<a class="bk_pop" href="#CDR0000784522_rl_909_1">1</a>,<a class="bk_pop" href="#CDR0000784522_rl_909_2">2</a>]</p><div id="CDR0000784522_rl_909"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_rl_909_1">Sultan I, Casanova M, Ferrari A, et al.: Differential features of nasopharyngeal carcinoma in children and adults: a SEER study. Pediatr Blood Cancer 55 (2): 279-84, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20582982" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20582982</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_909_2">Richards MK, Dahl JP, Gow K, et al.: Factors Associated With Mortality in Pediatric vs Adult Nasopharyngeal Carcinoma. JAMA Otolaryngol Head Neck Surg 142 (3): 217-22, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26769566" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26769566</span></a>]</div></li></ol></div></div><div id="CDR0000784522__911"><h2 id="_CDR0000784522__911_">Clinical Presentation</h2><p id="CDR0000784522__912">Signs and symptoms of nasopharyngeal carcinoma include the following:[<a class="bk_pop" href="#CDR0000784522_rl_911_1">1</a>,<a class="bk_pop" href="#CDR0000784522_rl_911_2">2</a>]</p><ul id="CDR0000784522__1237"><li class="half_rhythm"><div>Cervical lymphadenopathy.</div></li><li class="half_rhythm"><div>Nosebleeds.</div></li><li class="half_rhythm"><div>Nasal congestion and obstruction.</div></li><li class="half_rhythm"><div>Headache.</div></li><li class="half_rhythm"><div>Otalgia. </div></li><li class="half_rhythm"><div>Otitis media. </div></li></ul><p id="CDR0000784522__1238">Given the rich lymphatic drainage of the nasopharynx, bilateral cervical lymphadenopathy is often the first sign of disease. The tumor spreads locally to adjacent areas of the oropharynx and may invade the skull base, resulting in cranial nerve palsy or difficulty with movements of the jaw (trismus).</p><p id="CDR0000784522__1239">Distant metastatic sites may include the bones, lungs,
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and liver. </p><div id="CDR0000784522_rl_911"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_rl_911_1">Yan Z, Xia L, Huang Y, et al.: Nasopharyngeal carcinoma in children and adolescents in an endemic area: a report of 185 cases. Int J Pediatr Otorhinolaryngol 77 (9): 1454-60, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23830224" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23830224</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_911_2">Hu S, Xu X, Xu J, et al.: Prognostic factors and long-term outcomes of nasopharyngeal carcinoma in children and adolescents. Pediatr Blood Cancer 60 (7): 1122-7, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23303571" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23303571</span></a>]</div></li></ol></div></div><div id="CDR0000784522__913"><h2 id="_CDR0000784522__913_">Diagnostic and Staging Evaluation</h2><p id="CDR0000784522__914">Diagnostic tests will determine the extent of the primary tumor and the presence of metastases. Visualization of the nasopharynx by an otolaryngologist using nasal endoscopy and magnetic resonance
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imaging of the head and neck can be used to determine the extent of the
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primary tumor.</p><p id="CDR0000784522__1240">A diagnosis can be made from a biopsy of the
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primary tumor or enlarged lymph nodes of the neck. Nasopharyngeal carcinomas must be distinguished from all other cancers that can present with
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enlarged lymph nodes and from other types of cancer in the head and neck area.
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Thus, diseases such as thyroid cancer, rhabdomyosarcoma, non-Hodgkin
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lymphoma including Burkitt lymphoma, and Hodgkin lymphoma must be considered, as
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well as benign conditions such as nasal angiofibroma, which usually presents with
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epistaxis in adolescent males, infectious lymphadenitis, and Rosai-Dorfman disease.</p><p id="CDR0000784522__1241">Evaluation of the chest and abdomen by computed tomography (CT) and bone scan is performed to determine whether there is metastatic
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disease. Fluorine F 18-fludeoxyglucose positron emission tomography (PET)–CT may also be helpful in the evaluation of potential metastatic lesions.[<a class="bk_pop" href="#CDR0000784522_rl_913_1">1</a>]</p><div id="CDR0000784522_rl_913"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_rl_913_1">Cheuk DK, Sabin ND, Hossain M, et al.: PET/CT for staging and follow-up of pediatric nasopharyngeal carcinoma. Eur J Nucl Med Mol Imaging 39 (7): 1097-106, 2012. [<a href="/pmc/articles/PMC3531235/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3531235</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/22532252" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22532252</span></a>]</div></li></ol></div></div><div id="CDR0000784522__915"><h2 id="_CDR0000784522__915_">Stage Information for Childhood Nasopharyngeal Carcinoma</h2><p id="CDR0000784522__916">Tumor staging is performed using the <a href="https://www.cancer.gov/types/head-and-neck/hp/nasopharyngeal-treatment-pdq#section/_16" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">tumor-node-metastasis (TNM) classification system</a>
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of the American Joint Committee on Cancer.[<a class="bk_pop" href="#CDR0000784522_rl_915_1">1</a>,<a class="bk_pop" href="#CDR0000784522_rl_915_2">2</a>]</p><p id="CDR0000784522__1242">More than 90% of children and adolescents with nasopharyngeal carcinoma present with advanced disease (stage III/IV or T3/T4).[<a class="bk_pop" href="#CDR0000784522_rl_915_3">3</a>,<a class="bk_pop" href="#CDR0000784522_rl_915_4">4</a>] Population-based studies have reported that patients younger than 20 years had a higher incidence of advanced-stage disease than did adult patients.[<a class="bk_pop" href="#CDR0000784522_rl_915_5">5</a>,<a class="bk_pop" href="#CDR0000784522_rl_915_6">6</a>] However, less than 10% of children and adolescents with nasopharyngeal carcinoma presented with distant metastases at diagnosis.[<a class="bk_pop" href="#CDR0000784522_rl_915_3">3</a>,<a class="bk_pop" href="#CDR0000784522_rl_915_4">4</a>,<a class="bk_pop" href="#CDR0000784522_rl_915_7">7</a>]</p><div id="CDR0000784522_rl_915"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_rl_915_1">Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017.</div></li><li><div class="bk_ref" id="CDR0000784522_rl_915_2">Lee AWM, Lydiatt WM, Colevas AD, et al.: Nasopharynx. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 103-11.</div></li><li><div class="bk_ref" id="CDR0000784522_rl_915_3">Cheuk DK, Billups CA, Martin MG, et al.: Prognostic factors and long-term outcomes of childhood nasopharyngeal carcinoma. Cancer 117 (1): 197-206, 2011. [<a href="/pmc/articles/PMC2994981/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2994981</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20737561" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20737561</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_915_4">Casanova M, Bisogno G, Gandola L, et al.: A prospective protocol for nasopharyngeal carcinoma in children and adolescents: the Italian Rare Tumors in Pediatric Age (TREP) project. Cancer 118 (10): 2718-25, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/21918965" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21918965</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_915_5">Sultan I, Casanova M, Ferrari A, et al.: Differential features of nasopharyngeal carcinoma in children and adults: a SEER study. Pediatr Blood Cancer 55 (2): 279-84, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20582982" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20582982</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_915_6">Richards MK, Dahl JP, Gow K, et al.: Factors Associated With Mortality in Pediatric vs Adult Nasopharyngeal Carcinoma. JAMA Otolaryngol Head Neck Surg 142 (3): 217-22, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26769566" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26769566</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_915_7">Buehrlen M, Zwaan CM, Granzen B, et al.: Multimodal treatment, including interferon beta, of nasopharyngeal carcinoma in children and young adults: preliminary results from the prospective, multicenter study NPC-2003-GPOH/DCOG. Cancer 118 (19): 4892-900, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22359313" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22359313</span></a>]</div></li></ol></div></div><div id="CDR0000784522__917"><h2 id="_CDR0000784522__917_">Prognosis</h2><p id="CDR0000784522__918">The overall survival of children and adolescents with nasopharyngeal carcinoma has improved over the last four decades; with state-of-the-art multimodal treatment, 5-year survival rates exceed 80%.[<a class="bk_pop" href="#CDR0000784522_rl_917_1">1</a>-<a class="bk_pop" href="#CDR0000784522_rl_917_8">8</a>] After controlling for stage, children with nasopharyngeal carcinoma have significantly better outcomes than do adults.[<a class="bk_pop" href="#CDR0000784522_rl_917_1">1</a>,<a class="bk_pop" href="#CDR0000784522_rl_917_7">7</a>] However, the intensive use of chemotherapy and radiation therapy results in significant acute and long-term morbidities, including subsequent neoplasms.[<a class="bk_pop" href="#CDR0000784522_rl_917_1">1</a>-<a class="bk_pop" href="#CDR0000784522_rl_917_3">3</a>,<a class="bk_pop" href="#CDR0000784522_rl_917_6">6</a>]</p><div id="CDR0000784522_rl_917"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_rl_917_1">Sultan I, Casanova M, Ferrari A, et al.: Differential features of nasopharyngeal carcinoma in children and adults: a SEER study. Pediatr Blood Cancer 55 (2): 279-84, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20582982" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20582982</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_917_2">Cheuk DK, Billups CA, Martin MG, et al.: Prognostic factors and long-term outcomes of childhood nasopharyngeal carcinoma. Cancer 117 (1): 197-206, 2011. [<a href="/pmc/articles/PMC2994981/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2994981</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20737561" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20737561</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_917_3">Casanova M, Bisogno G, Gandola L, et al.: A prospective protocol for nasopharyngeal carcinoma in children and adolescents: the Italian Rare Tumors in Pediatric Age (TREP) project. Cancer 118 (10): 2718-25, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/21918965" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21918965</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_917_4">Buehrlen M, Zwaan CM, Granzen B, et al.: Multimodal treatment, including interferon beta, of nasopharyngeal carcinoma in children and young adults: preliminary results from the prospective, multicenter study NPC-2003-GPOH/DCOG. Cancer 118 (19): 4892-900, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22359313" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22359313</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_917_5">Hu S, Xu X, Xu J, et al.: Prognostic factors and long-term outcomes of nasopharyngeal carcinoma in children and adolescents. Pediatr Blood Cancer 60 (7): 1122-7, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23303571" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23303571</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_917_6">Sahai P, Mohanti BK, Sharma A, et al.: Clinical outcome and morbidity in pediatric patients with nasopharyngeal cancer treated with chemoradiotherapy. Pediatr Blood Cancer 64 (2): 259-266, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/27681956" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 27681956</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_917_7">Richards MK, Dahl JP, Gow K, et al.: Factors Associated With Mortality in Pediatric vs Adult Nasopharyngeal Carcinoma. JAMA Otolaryngol Head Neck Surg 142 (3): 217-22, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26769566" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26769566</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_917_8">Gioacchini FM, Tulli M, Kaleci S, et al.: Prognostic aspects in the treatment of juvenile nasopharyngeal carcinoma: a systematic review. Eur Arch Otorhinolaryngol 274 (3): 1205-1214, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/27318701" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 27318701</span></a>]</div></li></ol></div></div><div id="CDR0000784522__919"><h2 id="_CDR0000784522__919_">Treatment of Newly Diagnosed Childhood Nasopharyngeal Carcinoma</h2><p id="CDR0000784522__920">Treatment of nasopharyngeal carcinoma is multimodal and includes the following:</p><ol id="CDR0000784522__921"><li class="half_rhythm"><div><b>Combined-modality therapy with chemotherapy and radiation.</b> High-dose radiation therapy alone has a role in the management of nasopharyngeal carcinoma; however, studies in both children and adults show that combined-modality therapy with chemotherapy and radiation is the most effective way to treat nasopharyngeal carcinoma.<ol id="CDR0000784522__775" class="lower-alpha"><li class="half_rhythm"><div class="half_rhythm">Several studies have investigated the role of chemotherapy in the treatment of adult nasopharyngeal carcinoma. The use of concomitant chemoradiation therapy has been consistently associated with a significant survival benefit, including improved locoregional disease control and reduction in distant metastases.[<a class="bk_pop" href="#CDR0000784522_rl_919_1">1</a>-<a class="bk_pop" href="#CDR0000784522_rl_919_3">3</a>] The addition of neoadjuvant chemotherapy to concomitant chemoradiation has further improved outcomes, whereas the impact of adjuvant chemotherapy is less defined.[<a class="bk_pop" href="#CDR0000784522_rl_919_2">2</a>,<a class="bk_pop" href="#CDR0000784522_rl_919_3">3</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">In children, most studies have used neoadjuvant chemotherapy with cisplatin and 5-fluorouracil (5-FU) followed by concomitant chemoradiation with single-agent cisplatin.[<a class="bk_pop" href="#CDR0000784522_rl_919_4">4</a>-<a class="bk_pop" href="#CDR0000784522_rl_919_6">6</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335132/" class="def">Level of evidence: 2A</a>] Using this approach, 5-year overall survival (OS) estimates are consistently above 80%.[<a class="bk_pop" href="#CDR0000784522_rl_919_5">5</a>,<a class="bk_pop" href="#CDR0000784522_rl_919_6">6</a>]</div><div class="half_rhythm">The following two modifications of this approach have been investigated:<ul id="CDR0000784522__776"><li class="half_rhythm"><div>The NPC-2003-GPOH study included a 6-month maintenance therapy phase with interferon-beta, and reported a 30-month OS estimate of 97.1%.[<a class="bk_pop" href="#CDR0000784522_rl_919_5">5</a>]</div></li><li class="half_rhythm"><div>A randomized prospective trial compared cisplatin and 5-FU with cisplatin, 5-FU, and docetaxel.[<a class="bk_pop" href="#CDR0000784522_rl_919_6">6</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335125/" class="def">Level of evidence: 1iiA</a>] The addition of docetaxel was not associated with improved outcome.</div></li></ul></div></li><li class="half_rhythm"><div class="half_rhythm">While nasopharyngeal carcinoma is a very chemosensitive neoplasm, high radiation doses to the nasopharynx and neck (approximately 65–70 Gy) are required for optimal locoregional control.[<a class="bk_pop" href="#CDR0000784522_rl_919_1">1</a>-<a class="bk_pop" href="#CDR0000784522_rl_919_3">3</a>] However, in children, studies using neoadjuvant chemotherapy have shown that it is possible to reduce the radiation dose to 55 Gy to 60 Gy for good responders.[<a class="bk_pop" href="#CDR0000784522_rl_919_4">4</a>,<a class="bk_pop" href="#CDR0000784522_rl_919_5">5</a>]</div></li></ol></div></li><li class="half_rhythm"><div><b>Surgery.</b> Surgery has a limited role in the management of nasopharyngeal carcinoma; the disease is usually considered unresectable because of extensive local spread.</div></li></ol><p id="CDR0000784522__1875"> The combination of cisplatin-based chemotherapy and high doses of radiation therapy to the nasopharynx and neck are associated with a high probability of hearing loss, hypothyroidism and panhypopituitarism, trismus, xerostomia, dental problems, and chronic sinusitis or otitis.[<a class="bk_pop" href="#CDR0000784522_rl_919_4">4</a>,<a class="bk_pop" href="#CDR0000784522_rl_919_7">7</a>,<a class="bk_pop" href="#CDR0000784522_rl_919_8">8</a>]; [<a class="bk_pop" href="#CDR0000784522_rl_919_9">9</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335150/" class="def">Level of evidence: 3iiiA</a>] (Refer to the PDQ summary on <a href="/books/n/pdqcis/CDR0000343584/">Late Effects of Treatment for Childhood Cancer</a> for more information.)</p><div id="CDR0000784522_rl_919"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_rl_919_1">Langendijk JA, Leemans ChR, Buter J, et al.: The additional value of chemotherapy to radiotherapy in locally advanced nasopharyngeal carcinoma: a meta-analysis of the published literature. J Clin Oncol 22 (22): 4604-12, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15542811" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15542811</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_919_2">Yan M, Kumachev A, Siu LL, et al.: Chemoradiotherapy regimens for locoregionally advanced nasopharyngeal carcinoma: A Bayesian network meta-analysis. Eur J Cancer 51 (12): 1570-9, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/26044925" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26044925</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_919_3">Ribassin-Majed L, Marguet S, Lee AWM, et al.: What Is the Best Treatment of Locally Advanced Nasopharyngeal Carcinoma? An Individual Patient Data Network Meta-Analysis. J Clin Oncol 35 (5): 498-505, 2017. [<a href="/pmc/articles/PMC5791836/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5791836</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/27918720" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 27918720</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_919_4">Casanova M, Bisogno G, Gandola L, et al.: A prospective protocol for nasopharyngeal carcinoma in children and adolescents: the Italian Rare Tumors in Pediatric Age (TREP) project. Cancer 118 (10): 2718-25, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/21918965" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21918965</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_919_5">Buehrlen M, Zwaan CM, Granzen B, et al.: Multimodal treatment, including interferon beta, of nasopharyngeal carcinoma in children and young adults: preliminary results from the prospective, multicenter study NPC-2003-GPOH/DCOG. Cancer 118 (19): 4892-900, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22359313" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22359313</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_919_6">Casanova M, Özyar E, Patte C, et al.: International randomized phase 2 study on the addition of docetaxel to the combination of cisplatin and 5-fluorouracil in the induction treatment for nasopharyngeal carcinoma in children and adolescents. Cancer Chemother Pharmacol 77 (2): 289-98, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26666649" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26666649</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_919_7">Cheuk DK, Billups CA, Martin MG, et al.: Prognostic factors and long-term outcomes of childhood nasopharyngeal carcinoma. Cancer 117 (1): 197-206, 2011. [<a href="/pmc/articles/PMC2994981/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2994981</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20737561" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20737561</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_919_8">Sahai P, Mohanti BK, Sharma A, et al.: Clinical outcome and morbidity in pediatric patients with nasopharyngeal cancer treated with chemoradiotherapy. Pediatr Blood Cancer 64 (2): 259-266, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/27681956" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 27681956</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_919_9">Hu S, Xu X, Xu J, et al.: Prognostic factors and long-term outcomes of nasopharyngeal carcinoma in children and adolescents. Pediatr Blood Cancer 60 (7): 1122-7, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23303571" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23303571</span></a>]</div></li></ol></div></div><div id="CDR0000784522__1876"><h2 id="_CDR0000784522__1876_">Treatment of Refractory Childhood Nasopharyngeal Carcinoma</h2><p id="CDR0000784522__1877">Given the unique pathogenesis of nasopharyngeal carcinoma, immunotherapy has been explored for patients with refractory disease, as follows:</p><ul id="CDR0000784522__1878"><li class="half_rhythm"><div>The use of Epstein-Barr virus (EBV)–specific cytotoxic T-lymphocyte therapy has shown to be a very promising approach with minimal toxicity and evidence of significant antitumor activity in patients with relapsed or refractory nasopharyngeal carcinoma.[<a class="bk_pop" href="#CDR0000784522_rl_1876_1">1</a>] In a phase I/II study of EBV-specific cytotoxic T-lymphocyte therapy in patients with refractory disease, response rates were observed in 33.3% of patients, and long-term remissions were obtained in 62% of patients treated in their second or subsequent remission.[<a class="bk_pop" href="#CDR0000784522_rl_1876_2">2</a>]</div></li><li class="half_rhythm"><div>Anti–programmed death-ligand 1 (PD-L1) monoclonal antibodies have been studied in two phase II trials in adults with refractory nasopharyngeal carcinoma, with response rates of 20.5% to 25.9% (33% in patients with PD-L1–positive tumors) and evidence of long-term remissions.[<a class="bk_pop" href="#CDR0000784522_rl_1876_3">3</a>,<a class="bk_pop" href="#CDR0000784522_rl_1876_4">4</a>]</div></li></ul><div id="CDR0000784522_rl_1876"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_rl_1876_1">Straathof KC, Bollard CM, Popat U, et al.: Treatment of nasopharyngeal carcinoma with Epstein-Barr virus--specific T lymphocytes. Blood 105 (5): 1898-904, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15542583" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15542583</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_1876_2">Louis CU, Straathof K, Bollard CM, et al.: Adoptive transfer of EBV-specific T cells results in sustained clinical responses in patients with locoregional nasopharyngeal carcinoma. J Immunother 33 (9): 983-90, 2010 Nov-Dec. [<a href="/pmc/articles/PMC2964409/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2964409</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20948438" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20948438</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_1876_3">Hsu C, Lee SH, Ejadi S, et al.: Safety and Antitumor Activity of Pembrolizumab in Patients With Programmed Death-Ligand 1-Positive Nasopharyngeal Carcinoma: Results of the KEYNOTE-028 Study. J Clin Oncol 35 (36): 4050-4056, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28837405" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28837405</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_1876_4">Ma BBY, Lim WT, Goh BC, et al.: Antitumor Activity of Nivolumab in Recurrent and Metastatic Nasopharyngeal Carcinoma: An International, Multicenter Study of the Mayo Clinic Phase 2 Consortium (NCI-9742). J Clin Oncol 36 (14): 1412-1418, 2018. [<a href="/pmc/articles/PMC5941615/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5941615</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/29584545" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29584545</span></a>]</div></li></ol></div></div><div id="CDR0000784522__1470"><h2 id="_CDR0000784522__1470_">Treatment Options Under Clinical Evaluation for Childhood Nasopharyngeal Carcinoma</h2><p id="CDR0000784522__1873">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, refer to the <a href="https://clinicaltrials.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a>.</p><p id="CDR0000784522__1471">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000784522__1472"><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 using a next-generation sequencing targeted assay of more than 3,000 different mutations across more than 160 genes in refractory and recurrent solid tumors. Children and adolescents aged 1 to 21 years are eligible for the trial.</div><div class="half_rhythm">Tumor tissue from progressive or recurrent disease must be available for molecular characterization. Patients with tumors that have molecular variants addressed by treatment arms included in the trial will be offered treatment on Pediatric MATCH. Additional information can be obtained on the <a href="https://clinicaltrials.gov" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a> for <a href="https://www.cancer.gov/clinicaltrials/NCT03155620" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">APEC1621 (NCT03155620)</a>.
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</div></li></ul></div><div id="CDR0000784522__1864"><h2 id="_CDR0000784522__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000784522__1865">Cancer in children and adolescents is rare, although the overall incidence of childhood cancer has been slowly increasing since 1975.[<a class="bk_pop" href="#CDR0000784522_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 for children and adolescents with cancer. 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="CDR0000784522__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="CDR0000784522__1867"> (Refer to the PDQ <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> summaries for specific information about supportive care for children and adolescents with cancer.)</p><p id="CDR0000784522__1868">Guidelines for
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pediatric cancer centers and their role in the treatment of pediatric patients
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with cancer have been outlined by the American Academy of Pediatrics.[<a class="bk_pop" href="#CDR0000784522_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 in these trials 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 therapy that is currently
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accepted as standard. 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="CDR0000784522__1869">Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality decreased by more than 50%.[<a class="bk_pop" href="#CDR0000784522_rl_1864_3">3</a>] Childhood and adolescent cancer survivors require close monitoring because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ summary on <a href="/books/n/pdqcis/CDR0000343584/">Late Effects of Treatment for Childhood Cancer</a> for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)</p><p id="CDR0000784522__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="#CDR0000784522_rl_1864_4">4</a>] The U.S. <a href="https://history.nih.gov/research/downloads/PL107-280.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 persons. Therefore, all pediatric cancers are considered rare.</p><p id="CDR0000784522__1881">The designation of a rare tumor is not uniform among pediatric and adult groups. Adult rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people, and they are estimated to 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="#CDR0000784522_rl_1864_5">5</a>,<a class="bk_pop" href="#CDR0000784522_rl_1864_6">6</a>] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000784522__1871"><li class="half_rhythm"><div class="half_rhythm">The Italian cooperative project on rare pediatric tumors (Tumori Rari in Eta Pediatrica [TREP]) defines a pediatric rare tumor as one with an incidence of less than two cases per 1 million population per year and is not included in other clinical trials.[<a class="bk_pop" href="#CDR0000784522_rl_1864_7">7</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">The Children's Oncology Group has opted to define rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancer, melanoma and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinoma, nasopharyngeal carcinoma, and most adult-type carcinomas such as breast cancer, colorectal cancer, etc.).[<a class="bk_pop" href="#CDR0000784522_rl_1864_8">8</a>] These diagnoses account for about 4% of cancers diagnosed in children aged 0 to 14 years, compared with about 20% of cancers diagnosed in adolescents aged 15 to 19 years.[<a class="bk_pop" href="#CDR0000784522_rl_1864_9">9</a>]</div><div class="half_rhythm">Most cancers within subgroup XI are either melanomas or thyroid cancer, with the remaining subgroup XI cancer types accounting for only 1.3% of cancers in children aged 0 to 14 years and 5.3% of cancers in adolescents aged 15 to 19 years.</div></li></ul><p id="CDR0000784522__1872">These rare cancers are extremely challenging to study because of the low incidence 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><p id="CDR0000784522__1829">Information about these tumors may also be found in sources relevant to
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adults with cancer such as the PDQ summary on <a href="/books/n/pdqcis/CDR0000062918/">Nasopharyngeal Cancer Treatment (Adult)</a>.</p><div id="CDR0000784522_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000784522_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="CDR0000784522_rl_1864_2">Corrigan JJ, Feig SA; American Academy of Pediatrics: Guidelines for pediatric cancer centers. Pediatrics 113 (6): 1833-5, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15173520" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15173520</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_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="CDR0000784522_rl_1864_4">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="CDR0000784522_rl_1864_5">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="CDR0000784522_rl_1864_6">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="CDR0000784522_rl_1864_7">Ferrari A, Bisogno G, De Salvo GL, et al.: The challenge of very rare tumours in childhood: the Italian TREP project. Eur J Cancer 43 (4): 654-9, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17049226" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17049226</span></a>]</div></li><li><div class="bk_ref" id="CDR0000784522_rl_1864_8">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><li><div class="bk_ref" id="CDR0000784522_rl_1864_9">Howlader N, Noone AM, Krapcho M, et al., eds.: SEER Cancer Statistics Review, 1975-2012. Bethesda, Md: National Cancer Institute, 2015. <a href="http://seer.cancer.gov/csr/1975_2012/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Also available online</a>. Last accessed January 31, 2019.</div></li></ol></div></div><div id="CDR0000784522__1559"><h2 id="_CDR0000784522__1559_">Changes to This Summary (09/21/2018)</h2><p id="CDR0000784522__1560">The PDQ cancer information summaries are reviewed regularly and updated as
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new information becomes available. This section describes the latest
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changes made to this summary as of the date above.</p><p id="CDR0000784522__1879">This summary was comprehensively reviewed, extensively revised, and reformatted.</p><p id="CDR0000784522__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="#CDR0000784522__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® - NCI's Comprehensive Cancer Database</a> pages.
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</p></div><div id="CDR0000784522__AboutThis_1"><h2 id="_CDR0000784522__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000784522__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000784522__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood nasopharyngeal cancer. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.</p></div><div id="CDR0000784522__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000784522__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="CDR0000784522__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000784522__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="CDR0000784522__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 Nasopharyngeal Cancer 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 (Dana-Farber Cancer Institute/Boston Children's Hospital)</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>R Beverly Raney, MD (Consultant)</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="CDR0000784522__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="CDR0000784522__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000784522__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="CDR0000784522__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000784522__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="CDR0000784522__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000784522__AboutThis_15">PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Nasopharyngeal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/head-and-neck/hp/child/nasopharyngeal-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/head-and-neck/hp/child/nasopharyngeal-treatment-pdq</a>. Accessed <MM/DD/YYYY>. </p><p id="CDR0000784522__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="CDR0000784522__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000784522__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="CDR0000784522__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000784522__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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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="#CDR0000784522__905" ref="log$=inpage&link_id=inpage">Incidence</a></li><li><a href="#CDR0000784522__907" ref="log$=inpage&link_id=inpage">Risk Factors</a></li><li><a href="#CDR0000784522__909" ref="log$=inpage&link_id=inpage">Histology</a></li><li><a href="#CDR0000784522__911" ref="log$=inpage&link_id=inpage">Clinical Presentation</a></li><li><a href="#CDR0000784522__913" ref="log$=inpage&link_id=inpage">Diagnostic and Staging Evaluation</a></li><li><a href="#CDR0000784522__915" ref="log$=inpage&link_id=inpage">Stage Information for Childhood Nasopharyngeal Carcinoma</a></li><li><a href="#CDR0000784522__917" ref="log$=inpage&link_id=inpage">Prognosis</a></li><li><a href="#CDR0000784522__919" ref="log$=inpage&link_id=inpage">Treatment of Newly Diagnosed Childhood Nasopharyngeal Carcinoma</a></li><li><a href="#CDR0000784522__1876" ref="log$=inpage&link_id=inpage">Treatment of Refractory Childhood Nasopharyngeal Carcinoma</a></li><li><a href="#CDR0000784522__1470" ref="log$=inpage&link_id=inpage">Treatment Options Under Clinical Evaluation for Childhood Nasopharyngeal Carcinoma</a></li><li><a href="#CDR0000784522__1864" ref="log$=inpage&link_id=inpage">Special Considerations for the Treatment of Children With Cancer</a></li><li><a href="#CDR0000784522__1559" ref="log$=inpage&link_id=inpage">Changes to This Summary (09/21/2018)</a></li><li><a href="#CDR0000784522__AboutThis_1" ref="log$=inpage&link_id=inpage">About This PDQ Summary</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related publications</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="document-links" id="Shutter"></a></div><div class="portlet_content"><ul 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offscreen_noflow">Links to PubMed</div></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Similar articles in PubMed</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="PBooksDiscovery_RA" id="Shutter"></a></div><div class="portlet_content"><ul><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389243" ref="ordinalpos=1&linkpos=1&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/31909946" ref="ordinalpos=1&linkpos=2&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Melanoma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span 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