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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="#__NBK552280_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK552280_dtls__"><div>Bethesda (MD): <a href="http://www.cancer.gov/" ref="pagearea=page-banner&amp;targetsite=external&amp;targetcat=link&amp;targettype=publisher">National Cancer Institute (US)</a>; 2002-.</div></div><div class="half_rhythm"></div><div class="bk_noprnt"><form method="get" action="/books/n/pdqcis/" id="bk_srch"><div class="bk_search"><label for="bk_term" class="offscreen_noflow">Search term</label><input type="text" title="Search this book" id="bk_term" name="term" value="" data-jig="ncbiclearbutton" /> <input type="submit" class="jig-ncbibutton" value="Search this book" submit="false" style="padding: 0.1em 0.4em;" /></div></form></div></div></div></div></div>
<div class="main-content lit-style" itemscope="itemscope" itemtype="http://schema.org/CreativeWork"><div class="meta-content fm-sec"><h1 id="_NBK552280_"><span class="title" itemprop="name">Childhood Carcinoma of Unknown Primary Treatment (PDQ&#x000ae;)</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 30, 2022.</p><p class="small">Created: <span itemprop="datePublished">December 23, 2019</span>.</p></div><div class="jig-ncbiinpagenav body-content whole_rhythm" data-jigconfig="allHeadingLevels: ['h2'],smoothScroll: false" itemprop="text"><div id="_abs_rndgid_" itemprop="description"><p id="CDR0000800113__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric carcinoma of unknown primary. 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="CDR0000800113__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="CDR0000800113__1006"><h2 id="_CDR0000800113__1006_">Incidence and Clinical Presentation</h2><p id="CDR0000800113__1007">Cancers of unknown primary sites present as metastatic cancers for which precise primary tumor
sites cannot be determined.[<a class="bk_pop" href="#CDR0000800113_rl_1006_1">1</a>] As an example, lymph nodes at the base of the
skull may enlarge in relationship to a tumor on the face or scalp that is not evident by physical examination or radiographic imaging.
Thus, modern imaging techniques may indicate the extent of the disease but not
a primary site. Tumors such as adenocarcinomas, melanomas, and embryonal tumors, like
rhabdomyosarcomas and neuroblastomas, may present in this way.</p><p id="CDR0000800113__1319">Less than 1% of all solid cancers of unknown primary sites occur in children. Because of the age-related incidence of tumor types, embryonal histologies are more common in children.[<a class="bk_pop" href="#CDR0000800113_rl_1006_2">2</a>]</p><div id="CDR0000800113_rl_1006"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800113_rl_1006_1">Kuttesch JF, Parham DM, Kaste SC, et al.: Embryonal malignancies of unknown primary origin in children. Cancer 75 (1): 115-21, 1995. [<a href="https://pubmed.ncbi.nlm.nih.gov/7804965" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 7804965</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1006_2">Pavlidis N, Pentheroudakis G: Cancer of unknown primary site. Lancet 379 (9824): 1428-35, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22414598" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 22414598</span></a>]</div></li></ol></div></div><div id="CDR0000800113__1008"><h2 id="_CDR0000800113__1008_">Diagnostic Evaluation</h2><p id="CDR0000800113__1009">For all patients who present with tumors from unknown primary sites,
treatment is directed toward the specific histopathology of the tumor and
is age-appropriate for the general type of cancer suspected, regardless of
the sites involved.[<a class="bk_pop" href="#CDR0000800113_rl_1008_1">1</a>] </p><p id="CDR0000800113__1454">Studies in adults suggest that positron emission tomography (PET) imaging can be helpful in identifying cancers of unknown primary sites, particularly in patients whose tumors arise in the head and neck area.[<a class="bk_pop" href="#CDR0000800113_rl_1008_2">2</a>] A report in adults using fluorine F 18-fludeoxyglucose PET-computed tomography identified 42.5% of primary tumors in a group of cancers of unknown primary sites.[<a class="bk_pop" href="#CDR0000800113_rl_1008_3">3</a>] </p><p id="CDR0000800113__1455">The use of gene expression profiling and next-generation sequencing can enhance the ability to identify the putative tissue of origin and guide the selection of targeted agents for specific mutations.[<a class="bk_pop" href="#CDR0000800113_rl_1008_4">4</a>-
<a class="bk_pop" href="#CDR0000800113_rl_1008_8">8</a>]</p><p id="CDR0000800113__2461"> In a study of 200 adult patients with carcinomas of unknown primary sites, 125 had adenocarcinomas and 75 had carcinomas without features of adenocarcinoma. Genomic alterations were found in 96% of the cases. The most common alterations were <i>TP53</i> (55%), <i>KRAS</i> (20%), <i>CDKN2A</i> (19%), and <i>MYC</i> (12%). Clinically relevant and potentially actionable mutations included <i>KRAS</i> (20%), <i>CDKN2A</i> (19%), <i>MCL1</i> (10%), <i>PTEN</i> (7%), <i>PIK3CA</i> (9%), <i>ERBB2</i> (8%), <i>RICTOR</i> (6%), <i>BRAF</i> (6%), and <i>NF1</i> (4%). These findings suggest that genomic profiling can help identify potentially actionable targets, which could benefit patients clinically while reducing the complex, costly workup needed to search for a primary tumor site of origin.[<a class="bk_pop" href="#CDR0000800113_rl_1008_9">9</a>]</p><p id="CDR0000800113__2462"> Despite reports of precision medicine studies in pediatric oncology, none of them has described a case of carcinoma of unknown primary site with a defined or actionable genomic alteration.[<a class="bk_pop" href="#CDR0000800113_rl_1008_10">10</a>]</p><div id="CDR0000800113_rl_1008"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800113_rl_1008_1">Kuttesch JF, Parham DM, Kaste SC, et al.: Embryonal malignancies of unknown primary origin in children. Cancer 75 (1): 115-21, 1995. [<a href="https://pubmed.ncbi.nlm.nih.gov/7804965" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 7804965</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1008_2">Bohuslavizki KH, Klutmann S, Kr&#x000f6;ger S, et al.: FDG PET detection of unknown primary tumors. J Nucl Med 41 (5): 816-22, 2000. [<a href="https://pubmed.ncbi.nlm.nih.gov/10809197" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 10809197</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1008_3">Han A, Xue J, Hu M, et al.: Clinical value of 18F-FDG PET-CT in detecting primary tumor for patients with carcinoma of unknown primary. Cancer Epidemiol 36 (5): 470-5, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22504050" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 22504050</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1008_4">Tothill RW, Li J, Mileshkin L, et al.: Massively-parallel sequencing assists the diagnosis and guided treatment of cancers of unknown primary. J Pathol 231 (4): 413-23, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/24037760" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 24037760</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1008_5">Varadhachary GR, Talantov D, Raber MN, et al.: Molecular profiling of carcinoma of unknown primary and correlation with clinical evaluation. J Clin Oncol 26 (27): 4442-8, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18802157" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18802157</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1008_6">Fizazi K, Greco FA, Pavlidis N, et al.: Cancers of unknown primary site: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 26 (Suppl 5): v133-8, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/26314775" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 26314775</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1008_7">Greco FA, Lennington WJ, Spigel DR, et al.: Poorly differentiated neoplasms of unknown primary site: diagnostic usefulness of a molecular cancer classifier assay. Mol Diagn Ther 19 (2): 91-7, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/25758902" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 25758902</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1008_8">Gatalica Z, Millis SZ, Vranic S, et al.: Comprehensive tumor profiling identifies numerous biomarkers of drug response in cancers of unknown primary site: analysis of 1806 cases. Oncotarget 5 (23): 12440-7, 2014. [<a href="/pmc/articles/PMC4322997/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC4322997</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/25415047" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 25415047</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1008_9">Ross JS, Wang K, Gay L, et al.: Comprehensive Genomic Profiling of Carcinoma of Unknown Primary Site: New Routes to Targeted Therapies. JAMA Oncol 1 (1): 40-9, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/26182302" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 26182302</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_rl_1008_10">Mody RJ, Prensner JR, Everett J, et al.: Precision medicine in pediatric oncology: Lessons learned and next steps. Pediatr Blood Cancer 64 (3): , 2017. [<a href="/pmc/articles/PMC5683396/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC5683396</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/27748023" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 27748023</span></a>]</div></li></ol></div></div><div id="CDR0000800113__1864"><h2 id="_CDR0000800113__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000800113__1865">Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[<a class="bk_pop" href="#CDR0000800113_rl_1864_1">1</a>] Referral to medical centers with multidisciplinary teams of cancer specialists experienced in treating cancers that occur in childhood and adolescence should be considered. This multidisciplinary team approach incorporates the skills
of the following health care professionals and others to ensure that children receive treatment, supportive care, and rehabilitation
that will achieve optimal survival and quality of life:</p><ul id="CDR0000800113__1866"><li class="half_rhythm"><div>Primary care physicians.</div></li><li class="half_rhythm"><div>Pediatric surgeons.</div></li><li class="half_rhythm"><div>Radiation
oncologists.</div></li><li class="half_rhythm"><div>Pediatric medical oncologists/hematologists.</div></li><li class="half_rhythm"><div> Rehabilitation
specialists.</div></li><li class="half_rhythm"><div>Pediatric nurse specialists.</div></li><li class="half_rhythm"><div>Social workers.</div></li><li class="half_rhythm"><div>Child-life professionals.</div></li><li class="half_rhythm"><div>Psychologists.</div></li></ul><p id="CDR0000800113__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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Supportive and Palliative Care</a>.</p><p id="CDR0000800113__1868">The American Academy of Pediatrics has outlined guidelines for
pediatric cancer centers and their role in the treatment of pediatric patients
with cancer.[<a class="bk_pop" href="#CDR0000800113_rl_1864_2">2</a>] At
these pediatric cancer centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity
to participate is offered to most patients and their families. Clinical
trials for children and adolescents diagnosed with cancer are generally
designed to compare potentially better therapy with current standard therapy. Most of the progress made in identifying curative
therapy for childhood cancers has been achieved through clinical trials.
Information about ongoing clinical trials is available from the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>.</p><p id="CDR0000800113__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="#CDR0000800113_rl_1864_3">3</a>-<a class="bk_pop" href="#CDR0000800113_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="CDR0000800113__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="#CDR0000800113_rl_1864_6">6</a>] The U.S. <a href="https://www.congress.gov/107/plaws/publ280/PLAW-107publ280.pdf" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;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="CDR0000800113__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="#CDR0000800113_rl_1864_7">7</a>,<a class="bk_pop" href="#CDR0000800113_rl_1864_8">8</a>] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000800113__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="#CDR0000800113_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="#CDR0000800113_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="#CDR0000800113_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="CDR0000800113__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><p id="CDR0000800113__1837">Information about these tumors may also be found in sources relevant to
adults with cancer, such as <a href="/books/n/pdqcis/CDR0000062936/">Carcinoma of Unknown Primary Treatment</a>.</p><div id="CDR0000800113_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800113_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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 20404250</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Also available online</a>. Last accessed May 19, 2023.</div></li><li><div class="bk_ref" id="CDR0000800113_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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 24853691</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Available online</a>. Last accessed May 19, 2023.</div></li><li><div class="bk_ref" id="CDR0000800113_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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Available online</a>. Last accessed August 18, 2023.</div></li><li><div class="bk_ref" id="CDR0000800113_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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 24488779</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 28687376</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 28542893</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_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&#x000a0;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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 30785015</span></a>]</div></li><li><div class="bk_ref" id="CDR0000800113_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&amp;targetsite=entrez&amp;targetcat=link&amp;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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 20956621</span></a>]</div></li></ol></div></div><div id="CDR0000800113__1010"><h2 id="_CDR0000800113__1010_">Treatment of Childhood Carcinoma of Unknown Primary</h2><p id="CDR0000800113__1011">Chemotherapy, targeted therapy, and radiation therapy
may be used to treat childhood carcinomas of unknown primary sites. The appropriate and relevant treatments, according to the general category of carcinoma or
sarcoma (depending on the histological findings, symptoms, and extent of tumor),
are initiated as early as possible.[<a class="bk_pop" href="#CDR0000800113_rl_1010_1">1</a>]</p><p id="CDR0000800113__1469">For more
information, see <a href="/books/n/pdqcis/CDR0000062936/">Carcinoma of Unknown Primary Treatment</a>.</p><div id="CDR0000800113_rl_1010"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000800113_rl_1010_1">Morris GJ, Greco FA, Hainsworth JD, et al.: Cancer of unknown primary site. Semin Oncol 37 (2): 71-9, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20494696" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 20494696</span></a>]</div></li></ol></div></div><div id="CDR0000800113__2013"><h2 id="_CDR0000800113__2013_">Treatment Options Under Clinical Evaluation for Childhood Carcinoma of Unknown Primary</h2><p id="CDR0000800113__2014">Information about National Cancer Institute (NCI)&#x02013;supported clinical trials can be found on the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/search" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a>. For information about clinical trials sponsored by other organizations, see the <a href="https://clinicaltrials.gov/" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">ClinicalTrials.gov website</a>.</p><p id="CDR0000800113__2015">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000800113__2016"><li class="half_rhythm"><div class="half_rhythm"><b><a href="https://www.cancer.gov/clinicaltrials/NCT03155620" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">NCI website</a> and <a href="https://clinicaltrials.gov/ct2/show/NCT03155620" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">ClinicalTrials.gov website</a>.
</div></li></ul></div><div id="CDR0000800113__2018"><h2 id="_CDR0000800113__2018_">Latest Updates to This Summary (12/30/2022)</h2><p id="CDR0000800113__2019">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="CDR0000800113__2463">This summary was reformatted.</p><p id="CDR0000800113__2460">
<b>
<a href="#CDR0000800113__1864">Special Considerations for the Treatment of Children With Cancer</a>
</b>
</p><p id="CDR0000800113__2457">Revised <a href="#CDR0000800113__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="CDR0000800113__2458">Revised <a href="/books/NBK552280.6/#CDR0000800113__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="CDR0000800113__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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">PDQ Pediatric Treatment Editorial Board</a>, which is
editorially independent of NCI. The summary reflects an independent review of
the literature and does not represent a policy statement of NCI or NIH. More
information about summary policies and the role of the PDQ Editorial Boards in
maintaining the PDQ summaries can be found on the <a href="#CDR0000800113__AboutThis_1">About This PDQ Summary</a> and <a href="https://www.cancer.gov/publications/pdq" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">PDQ&#x000ae; Cancer Information for Health Professionals</a> pages.
</p></div><div id="CDR0000800113__AboutThis_1"><h2 id="_CDR0000800113__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000800113__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000800113__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric carcinoma of unknown primary. 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="CDR0000800113__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000800113__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&amp;targetsite=external&amp;targetcat=link&amp;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="CDR0000800113__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000800113__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="CDR0000800113__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 Carcinoma of Unknown Primary 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="CDR0000800113__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&amp;targetsite=external&amp;targetcat=link&amp;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="CDR0000800113__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000800113__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="CDR0000800113__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000800113__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 &#x0201c;NCI&#x02019;s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].&#x0201d;</p><p id="CDR0000800113__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000800113__AboutThis_15">PDQ&#x000ae; Pediatric Treatment Editorial Board. PDQ Childhood Carcinoma of Unknown Primary Treatment. Bethesda, MD: National Cancer Institute. Updated &#x0003c;MM/DD/YYYY&#x0003e;. Available at: <a href="https://www.cancer.gov/types/unknown-primary/hp/child-unknown-primary-treatment-pdq" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">https://www.cancer.gov/types/unknown-primary/hp/child-unknown-primary-treatment-pdq</a>. Accessed &#x0003c;MM/DD/YYYY&#x0003e;. [PMID: 31909936]</p><p id="CDR0000800113__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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Visuals Online</a>, a collection of over 2,000 scientific images.
</p></div><div id="CDR0000800113__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000800113__AboutThis_18">Based on the strength of the available evidence, treatment options may be described as either &#x0201c;standard&#x0201d; or &#x0201c;under clinical evaluation.&#x0201d; 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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Managing Cancer Care</a> page.</p></div><div id="CDR0000800113__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000800113__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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Contact Us for Help</a> page. Questions can also be submitted to Cancer.gov through the website&#x02019;s <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Email Us</a>.</p></div></div></div></div>
<div class="post-content"><div><div class="half_rhythm"><a href="/books/about/copyright/">Copyright Notice</a></div><div class="small"><span class="label">Bookshelf ID: NBK552280</span><span class="label">PMID: <a href="https://pubmed.ncbi.nlm.nih.gov/31909936" title="PubMed record of this page" ref="pagearea=meta&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">31909936</a></span></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/NBK552280.6/?report=reader">PubReader</a></li><li><a href="/books/NBK552280.6/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK552280" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK552280" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Pediatric Treatment Editorial Board. Childhood Carcinoma of Unknown Primary Treatment (PDQ®): Health Professional Version. 2022 Dec 30. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. <span class="bk_cite_avail"></span></div></div></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/NBK552280.9/">NBK552280.9</a></span> August 13, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK552280.8/">NBK552280.8</a></span> December 8, 2023</li><li><span class="bk_col_itm"><a href="/books/NBK552280.7/">NBK552280.7</a></span> November 16, 2023</li><li><span class="bk_col_itm">NBK552280.6</span> December 30, 2022 (Displayed 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