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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="#__NBK549125_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK549125_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="_NBK549125_"><span class="title" itemprop="name">Childhood Colorectal Cancer 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: October 22, 2019.</p><p class="small">Created: <span itemprop="datePublished">October 22, 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="CDR0000799717__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric colorectal 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="CDR0000799717__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="CDR0000799717__657"><h2 id="_CDR0000799717__657_">Incidence</h2><p id="CDR0000799717__658">Carcinoma of the large bowel is rare in the pediatric age group.[<a class="bk_pop" href="#CDR0000799717_rl_657_1">1</a>] It is seen in one case per 1 million persons younger than 20 years in the United States annually; fewer than 100 cases are diagnosed in children each year in the United States.[<a class="bk_pop" href="#CDR0000799717_rl_657_2">2</a>] From 1973 to 2006, the Surveillance, Epidemiology, and End Results (SEER) database recorded 174 cases of colorectal cancer in patients younger than 19 years.[<a class="bk_pop" href="#CDR0000799717_rl_657_3">3</a>] Colorectal carcinoma accounts for about 2% of all malignancies in patients aged 15 to 29 years.[<a class="bk_pop" href="#CDR0000799717_rl_657_4">4</a>]</p><div id="CDR0000799717_rl_657"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799717_rl_657_1">da Costa Vieira RA, Tramonte MS, Lopes LF: Colorectal carcinoma in the first decade of life: a systematic review. Int J Colorectal Dis 30 (8): 1001-6, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/25916603" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 25916603</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_657_2">Saab R, Furman WL: Epidemiology and management options for colorectal cancer in children. Paediatr Drugs 10 (3): 177-92, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18454570" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18454570</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_657_3">Ferrari A, Casanova M, Massimino M, et al.: Peculiar features and tailored management of adult cancers occurring in pediatric age. Expert Rev Anticancer Ther 10 (11): 1837-51, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/21080807" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 21080807</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_657_4">Bleyer A, O&#x02019;Leary M, Barr R, et al., eds.: Cancer Epidemiology in Older Adolescents and Young Adults 15 to 29 Years of Age, Including SEER Incidence and Survival: 1975-2000. Bethesda, Md: National Cancer Institute, 2006. NIH Pub. No. 06-5767. <a href="https://seer.cancer.gov/archive/publications/aya/" ref="pagearea=cite-ref&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Also available online</a>. Last accessed August 16, 2019.</div></li></ol></div></div><div id="CDR0000799717__1162"><h2 id="_CDR0000799717__1162_">Clinical Presentation</h2><p id="CDR0000799717__1164">Colorectal tumors can occur in any location in the large bowel. Larger series and reviews suggest that ascending and descending colon tumors are each seen in approximately 30% of cases, with rectal tumors occurring in approximately 25% of cases.[<a class="bk_pop" href="#CDR0000799717_rl_1162_1">1</a>-<a class="bk_pop" href="#CDR0000799717_rl_1162_3">3</a>]</p><p id="CDR0000799717__1163">Signs and symptoms in children with descending colon tumors include the following:</p><ul id="CDR0000799717__1419"><li class="half_rhythm"><div>Abdominal pain (most common).</div></li><li class="half_rhythm"><div>Rectal bleeding.</div></li><li class="half_rhythm"><div>Change in bowel habits.</div></li><li class="half_rhythm"><div>Weight loss.</div></li><li class="half_rhythm"><div>Nausea and vomiting.</div></li></ul><p id="CDR0000799717__1420">The median duration of symptoms before diagnosis was about 3 months in one series.[<a class="bk_pop" href="#CDR0000799717_rl_1162_4">4</a>,<a class="bk_pop" href="#CDR0000799717_rl_1162_5">5</a>]</p><p id="CDR0000799717__1421">Changes in bowel habits may be associated with tumors of the rectum or lower colon.</p><p id="CDR0000799717__1422">Tumors of the right colon may cause more subtle symptoms but are often associated with the following:</p><ul id="CDR0000799717__1423"><li class="half_rhythm"><div>Abdominal mass.</div></li><li class="half_rhythm"><div>Weight loss.</div></li><li class="half_rhythm"><div>Decreased appetite.</div></li><li class="half_rhythm"><div>Blood in the stool</div></li><li class="half_rhythm"><div>Iron-deficiency anemia. </div></li></ul><p id="CDR0000799717__1424">Any tumor that causes complete obstruction of the large bowel can cause bowel perforation and spread of the tumor cells within the abdominal cavity.</p><div id="CDR0000799717_rl_1162"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799717_rl_1162_1">Kaplan MA, Isikdogan A, Gumus M, et al.: Childhood, adolescents, and young adults (&#x02264;25 y) colorectal cancer: study of Anatolian Society of Medical Oncology. J Pediatr Hematol Oncol 35 (2): 83-9, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23337551" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 23337551</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1162_2">Kim G, Baik SH, Lee KY, et al.: Colon carcinoma in childhood: review of the literature with four case reports. Int J Colorectal Dis 28 (2): 157-64, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23099637" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 23099637</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1162_3">Sultan I, Rodriguez-Galindo C, El-Taani H, et al.: Distinct features of colorectal cancer in children and adolescents: a population-based study of 159 cases. Cancer 116 (3): 758-65, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/19957323" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 19957323</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1162_4">Hill DA, Furman WL, Billups CA, et al.: Colorectal carcinoma in childhood and adolescence: a clinicopathologic review. J Clin Oncol 25 (36): 5808-14, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/18089879" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18089879</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1162_5">Saab R, Furman WL: Epidemiology and management options for colorectal cancer in children. Paediatr Drugs 10 (3): 177-92, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18454570" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18454570</span></a>]</div></li></ol></div></div><div id="CDR0000799717__1165"><h2 id="_CDR0000799717__1165_">Diagnostic Evaluation</h2><p id="CDR0000799717__1166">Diagnostic studies include the following:[<a class="bk_pop" href="#CDR0000799717_rl_1165_1">1</a>,<a class="bk_pop" href="#CDR0000799717_rl_1165_2">2</a>]</p><ul id="CDR0000799717__1287"><li class="half_rhythm"><div>Examination of the stool for blood.</div></li><li class="half_rhythm"><div>Studies of liver and kidney function.</div></li><li class="half_rhythm"><div>Measurement of carcinoembryonic antigen (CEA).</div></li><li class="half_rhythm"><div>Various medical imaging studies, including direct examination using colonoscopy to detect polyps in the large bowel. Other conventional radiographic studies include barium enema or video-capsule endoscopy followed by computed tomography of the chest and bone scans.[<a class="bk_pop" href="#CDR0000799717_rl_1165_3">3</a>]</div></li></ul><div id="CDR0000799717_rl_1165"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799717_rl_1165_1">Pratt CB, Rao BN, Merchant TE, et al.: Treatment of colorectal carcinoma in adolescents and young adults with surgery, 5-fluorouracil/leucovorin/interferon-alpha 2a and radiation therapy. Med Pediatr Oncol 32 (6): 459-60, 1999. [<a href="https://pubmed.ncbi.nlm.nih.gov/10358711" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 10358711</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1165_2">Kauffman WM, Jenkins JJ, Helton K, et al.: Imaging features of ovarian metastases from colonic adenocarcinoma in adolescents. Pediatr Radiol 25 (4): 286-8, 1995. [<a href="https://pubmed.ncbi.nlm.nih.gov/7567240" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 7567240</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1165_3">Postgate A, Hyer W, Phillips R, et al.: Feasibility of video capsule endoscopy in the management of children with Peutz-Jeghers syndrome: a blinded comparison with barium enterography for the detection of small bowel polyps. J Pediatr Gastroenterol Nutr 49 (4): 417-23, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19543117" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 19543117</span></a>]</div></li></ol></div></div><div id="CDR0000799717__659"><h2 id="_CDR0000799717__659_">Histology and Molecular Features</h2><p id="CDR0000799717__661">There is a higher incidence of mucinous adenocarcinoma in the pediatric and adolescent age group (40%&#x02013;50%), with many lesions being the signet ring cell type,[<a class="bk_pop" href="#CDR0000799717_rl_659_1">1</a>-<a class="bk_pop" href="#CDR0000799717_rl_659_5">5</a>] whereas only about 15% of adult lesions are of this histology. The tumors of younger patients with this histologic variant may be less responsive to chemotherapy. In the adolescent and young adult population with the mucinous histology, there is a higher incidence of signet ring cells, microsatellite instability, and mutations in the mismatch repair genes.[<a class="bk_pop" href="#CDR0000799717_rl_659_5">5</a>-<a class="bk_pop" href="#CDR0000799717_rl_659_7">7</a>] Tumors with mucinous histology arise from the surface of the bowel, usually at the site of an adenomatous polyp. The tumor may extend into the muscle layer surrounding the bowel, or the tumor may perforate the bowel entirely and seed through the spaces around the bowel, including intra-abdominal fat, lymph nodes, liver, ovaries, and the surface of other loops of bowel. A high incidence of metastasis involving the pelvis, ovaries, or both may be present in girls.[<a class="bk_pop" href="#CDR0000799717_rl_659_8">8</a>]</p><p id="CDR0000799717__1288">Colorectal cancers in younger patients with noninherited sporadic tumors often lack <i>KRAS</i> mutations and other cytogenetic anomalies seen in older patients.[<a class="bk_pop" href="#CDR0000799717_rl_659_9">9</a>] In a genomic study that used exome and RNA sequencing to identify mutational differences in colorectal carcinomas of adults (n = 30), adolescents and young adults (n = 30), and children (n = 2), five genes (<i>MYCBP2</i>, <i>BRCA2</i>, <i>PHLPP1</i>, <i>TOPORS</i>, and <i>ATR</i>) were identified that were more frequently mutated in adolescents and young adult patients. These genes contained a damaging mutation and were identified through whole-exome sequencing and RNA sequencing. In addition, higher mutational rates in DNA mismatch and DNA repair pathways, such as <i>MSH2</i>, <i>BRCA2</i>, and <i>RAD9B</i>, were more prevalent in adolescent and young adult samples but the results were not validated by RNA sequencing.[<a class="bk_pop" href="#CDR0000799717_rl_659_10">10</a>]</p><div id="CDR0000799717_rl_659"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799717_rl_659_1">Saab R, Furman WL: Epidemiology and management options for colorectal cancer in children. Paediatr Drugs 10 (3): 177-92, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18454570" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18454570</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_659_2">Hill DA, Furman WL, Billups CA, et al.: Colorectal carcinoma in childhood and adolescence: a clinicopathologic review. J Clin Oncol 25 (36): 5808-14, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/18089879" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18089879</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_659_3">Ferrari A, Rognone A, Casanova M, et al.: Colorectal carcinoma in children and adolescents: the experience of the Istituto Nazionale Tumori of Milan, Italy. Pediatr Blood Cancer 50 (3): 588-93, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/17405155" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 17405155</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_659_4">da Costa Vieira RA, Tramonte MS, Lopes LF: Colorectal carcinoma in the first decade of life: a systematic review. Int J Colorectal Dis 30 (8): 1001-6, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/25916603" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 25916603</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_659_5">Poles GC, Clark DE, Mayo SW, et al.: Colorectal carcinoma in pediatric patients: A comparison with adult tumors, treatment and outcomes from the National Cancer Database. J Pediatr Surg 51 (7): 1061-6, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26703433" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 26703433</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_659_6">Tricoli JV, Seibel NL, Blair DG, et al.: Unique characteristics of adolescent and young adult acute lymphoblastic leukemia, breast cancer, and colon cancer. J Natl Cancer Inst 103 (8): 628-35, 2011. [<a href="/pmc/articles/PMC3079851/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC3079851</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/21436065" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 21436065</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_659_7">Khan SA, Morris M, Idrees K, et al.: Colorectal cancer in the very young: a comparative study of tumor markers, pathology and survival in early onset and adult onset patients. J Pediatr Surg 51 (11): 1812-1817, 2016. [<a href="/pmc/articles/PMC5312708/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC5312708</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/27558481" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 27558481</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_659_8">Kauffman WM, Jenkins JJ, Helton K, et al.: Imaging features of ovarian metastases from colonic adenocarcinoma in adolescents. Pediatr Radiol 25 (4): 286-8, 1995. [<a href="https://pubmed.ncbi.nlm.nih.gov/7567240" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 7567240</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_659_9">Bleyer A, Barr R, Hayes-Lattin B, et al.: The distinctive biology of cancer in adolescents and young adults. Nat Rev Cancer 8 (4): 288-98, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18354417" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18354417</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_659_10">Tricoli JV, Boardman LA, Patidar R, et al.: A mutational comparison of adult and adolescent and young adult (AYA) colon cancer. Cancer 124 (5): 1070-1082, 2018. [<a href="/pmc/articles/PMC5821537/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC5821537</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/29194591" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 29194591</span></a>]</div></li></ol></div></div><div id="CDR0000799717__1515"><h2 id="_CDR0000799717__1515_">Staging</h2><p id="CDR0000799717__1167">Most reports also suggest that children present with more advanced disease than do adults, with 80% to 90% of patients presenting with Dukes stage C/D or TNM stage III/IV disease (refer to the <a href="/books/n/pdqcis/CDR0000062687/#CDR0000062687__39">Stage Information for Colon Cancer</a> section of the PDQ summary on adult <a href="/books/n/pdqcis/CDR0000062687/">Colon Cancer Treatment</a> for more information about staging).[<a class="bk_pop" href="#CDR0000799717_rl_1515_1">1</a>-<a class="bk_pop" href="#CDR0000799717_rl_1515_15">15</a>]</p><div id="CDR0000799717_rl_1515"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799717_rl_1515_1">Hill DA, Furman WL, Billups CA, et al.: Colorectal carcinoma in childhood and adolescence: a clinicopathologic review. J Clin Oncol 25 (36): 5808-14, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/18089879" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18089879</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_2">Saab R, Furman WL: Epidemiology and management options for colorectal cancer in children. Paediatr Drugs 10 (3): 177-92, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18454570" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18454570</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_3">Chantada GL, Perelli VB, Lombardi MG, et al.: Colorectal carcinoma in children, adolescents, and young adults. J Pediatr Hematol Oncol 27 (1): 39-41, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15654277" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 15654277</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_4">Durno C, Aronson M, Bapat B, et al.: Family history and molecular features of children, adolescents, and young adults with colorectal carcinoma. Gut 54 (8): 1146-50, 2005. [<a href="/pmc/articles/PMC1774876/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC1774876</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/15845562" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 15845562</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_5">Ferrari A, Rognone A, Casanova M, et al.: Colorectal carcinoma in children and adolescents: the experience of the Istituto Nazionale Tumori of Milan, Italy. Pediatr Blood Cancer 50 (3): 588-93, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/17405155" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 17405155</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_6">Karnak I, Ciftci AO, Senocak ME, et al.: Colorectal carcinoma in children. J Pediatr Surg 34 (10): 1499-504, 1999. [<a href="https://pubmed.ncbi.nlm.nih.gov/10549756" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 10549756</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_7">LaQuaglia MP, Heller G, Filippa DA, et al.: Prognostic factors and outcome in patients 21 years and under with colorectal carcinoma. J Pediatr Surg 27 (8): 1085-9; discussion 1089-90, 1992. [<a href="https://pubmed.ncbi.nlm.nih.gov/1403541" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 1403541</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_8">Radhakrishnan CN, Bruce J: Colorectal cancers in children without any predisposing factors. A report of eight cases and review of the literature. Eur J Pediatr Surg 13 (1): 66-8, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12664421" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 12664421</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_9">Sharma AK, Gupta CR: Colorectal cancer in children: case report and review of literature. Trop Gastroenterol 22 (1): 36-9, 2001 Jan-Mar. [<a href="https://pubmed.ncbi.nlm.nih.gov/11398246" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 11398246</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_10">Taguchi T, Suita S, Hirata Y, et al.: Carcinoma of the colon in children: a case report and review of 41 Japanese cases. J Pediatr Gastroenterol Nutr 12 (3): 394-9, 1991. [<a href="https://pubmed.ncbi.nlm.nih.gov/2072235" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 2072235</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_11">Pratt CB, Rao BN, Merchant TE, et al.: Treatment of colorectal carcinoma in adolescents and young adults with surgery, 5-fluorouracil/leucovorin/interferon-alpha 2a and radiation therapy. Med Pediatr Oncol 32 (6): 459-60, 1999. [<a href="https://pubmed.ncbi.nlm.nih.gov/10358711" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 10358711</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_12">Sultan I, Rodriguez-Galindo C, El-Taani H, et al.: Distinct features of colorectal cancer in children and adolescents: a population-based study of 159 cases. Cancer 116 (3): 758-65, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/19957323" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 19957323</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_13">Kaplan MA, Isikdogan A, Gumus M, et al.: Childhood, adolescents, and young adults (&#x02264;25 y) colorectal cancer: study of Anatolian Society of Medical Oncology. J Pediatr Hematol Oncol 35 (2): 83-9, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23337551" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 23337551</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_14">Kim G, Baik SH, Lee KY, et al.: Colon carcinoma in childhood: review of the literature with four case reports. Int J Colorectal Dis 28 (2): 157-64, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23099637" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 23099637</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1515_15">Poles GC, Clark DE, Mayo SW, et al.: Colorectal carcinoma in pediatric patients: A comparison with adult tumors, treatment and outcomes from the National Cancer Database. J Pediatr Surg 51 (7): 1061-6, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26703433" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 26703433</span></a>]</div></li></ol></div></div><div id="CDR0000799717__1168"><h2 id="_CDR0000799717__1168_">Treatment and Outcome of Childhood Colorectal Cancer</h2><p id="CDR0000799717__1169">Most patients present with evidence of metastatic disease,[<a class="bk_pop" href="#CDR0000799717_rl_1168_1">1</a>] either as gross
tumor or as microscopic deposits in lymph nodes, on the surface of the bowel,
or on intra-abdominal organs.[<a class="bk_pop" href="#CDR0000799717_rl_1168_2">2</a>,<a class="bk_pop" href="#CDR0000799717_rl_1168_3">3</a>] Of almost 160,000 patients with colorectal cancer included in the National Cancer Database, 918 pediatric patients were identified. Age younger than 21 years was a significant predictor of increased mortality.[<a class="bk_pop" href="#CDR0000799717_rl_1168_4">4</a>]</p><p id="CDR0000799717__1425">Treatment options for childhood colorectal cancer include the following:</p><ol id="CDR0000799717__1289"><li class="half_rhythm"><div class="half_rhythm"><b>Surgery:</b> Complete surgical excision is the most important prognostic factor and is the primary goal of surgery, but in most instances, this is impossible. Removal of large
portions of tumor provides little benefit for those with extensive
metastatic disease.[<a class="bk_pop" href="#CDR0000799717_rl_1168_5">5</a>] Most patients with microscopic metastatic disease
generally develop gross metastatic disease, and few individuals with metastatic
disease at diagnosis become long-term survivors.</div></li><li class="half_rhythm"><div class="half_rhythm"><b>Radiation therapy and chemotherapy:</b> Current therapy includes the use of radiation for rectal and lower colon
tumors, in conjunction with chemotherapy using 5-fluorouracil (5-FU) with
leucovorin.[<a class="bk_pop" href="#CDR0000799717_rl_1168_6">6</a>] Other agents, including irinotecan, may be of value.[<a class="bk_pop" href="#CDR0000799717_rl_1168_1">1</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335150/" class="def">Level of evidence: 3iiiA</a>] No significant benefit has been determined for interferon-alfa
given in conjunction with 5-FU/leucovorin.[<a class="bk_pop" href="#CDR0000799717_rl_1168_7">7</a>] </div><div class="half_rhythm">A recent review of nine clinical trials comprising 138 patients younger than 40 years demonstrated that the use of combination chemotherapy improved progression-free survival and overall survival (OS) in these patients. Furthermore, OS and response rates to chemotherapy were similar to those observed in older patients.[<a class="bk_pop" href="#CDR0000799717_rl_1168_8">8</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335132/" class="def">Level of evidence: 2A</a>] </div><div class="half_rhythm">Ipilimumab and nivolumab demonstrated high response rates in pediatric patients aged 12 years and older with microsatellite instability&#x02013;high or mismatch repair&#x02013;deficient metastatic colorectal cancer who had disease progression after treatment with a fluoropyrimidine, oxaliplatin, and irinotecan.[<a class="bk_pop" href="#CDR0000799717_rl_1168_9">9</a>]</div><div class="half_rhythm">Other active agents used in adults include oxaliplatin, bevacizumab, panitumumab, cetuximab, aflibercept, and regorafenib.[<a class="bk_pop" href="#CDR0000799717_rl_1168_10">10</a>-
<a class="bk_pop" href="#CDR0000799717_rl_1168_13">13</a>]</div></li></ol><p id="CDR0000799717__1171">Survival is consistent with the advanced stage of disease observed in most children with colorectal cancer, with an overall mortality rate of approximately 70%. For patients with a complete surgical resection or for those with low-stage/localized disease, survival is significantly prolonged, with the potential for cure.[<a class="bk_pop" href="#CDR0000799717_rl_1168_14">14</a>]</p><div id="CDR0000799717_rl_1168"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799717_rl_1168_1">Hill DA, Furman WL, Billups CA, et al.: Colorectal carcinoma in childhood and adolescence: a clinicopathologic review. J Clin Oncol 25 (36): 5808-14, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/18089879" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18089879</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_2">Ferrari A, Rognone A, Casanova M, et al.: Colorectal carcinoma in children and adolescents: the experience of the Istituto Nazionale Tumori of Milan, Italy. Pediatr Blood Cancer 50 (3): 588-93, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/17405155" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 17405155</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_3">Chantada GL, Perelli VB, Lombardi MG, et al.: Colorectal carcinoma in children, adolescents, and young adults. J Pediatr Hematol Oncol 27 (1): 39-41, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15654277" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 15654277</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_4">Poles GC, Clark DE, Mayo SW, et al.: Colorectal carcinoma in pediatric patients: A comparison with adult tumors, treatment and outcomes from the National Cancer Database. J Pediatr Surg 51 (7): 1061-6, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26703433" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 26703433</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_5">Saab R, Furman WL: Epidemiology and management options for colorectal cancer in children. Paediatr Drugs 10 (3): 177-92, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18454570" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18454570</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_6">Madajewicz S, Petrelli N, Rustum YM, et al.: Phase I-II trial of high-dose calcium leucovorin and 5-fluorouracil in advanced colorectal cancer. Cancer Res 44 (10): 4667-9, 1984. [<a href="https://pubmed.ncbi.nlm.nih.gov/6331882" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 6331882</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_7">Wolmark N, Bryant J, Smith R, et al.: Adjuvant 5-fluorouracil and leucovorin with or without interferon alfa-2a in colon carcinoma: National Surgical Adjuvant Breast and Bowel Project protocol C-05. J Natl Cancer Inst 90 (23): 1810-6, 1998. [<a href="https://pubmed.ncbi.nlm.nih.gov/9839521" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 9839521</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_8">Blanke CD, Bot BM, Thomas DM, et al.: Impact of young age on treatment efficacy and safety in advanced colorectal cancer: a pooled analysis of patients from nine first-line phase III chemotherapy trials. J Clin Oncol 29 (20): 2781-6, 2011. [<a href="/pmc/articles/PMC4874194/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC4874194</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/21646604" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 21646604</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_9">Overman MJ, Lonardi S, Wong KYM, et al.: Durable Clinical Benefit With Nivolumab Plus Ipilimumab in DNA Mismatch Repair-Deficient/Microsatellite Instability-High Metastatic Colorectal Cancer. J Clin Oncol 36 (8): 773-779, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/29355075" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 29355075</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_10">Saltz LB, Clarke S, D&#x000ed;az-Rubio E, et al.: Bevacizumab in combination with oxaliplatin-based chemotherapy as first-line therapy in metastatic colorectal cancer: a randomized phase III study. J Clin Oncol 26 (12): 2013-9, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18421054" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 18421054</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_11">Heinemann V, von Weikersthal LF, Decker T, et al.: FOLFIRI plus cetuximab versus FOLFIRI plus bevacizumab as first-line treatment for patients with metastatic colorectal cancer (FIRE-3): a randomised, open-label, phase 3 trial. Lancet Oncol 15 (10): 1065-75, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/25088940" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 25088940</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_12">Van Cutsem E, Tabernero J, Lakomy R, et al.: Addition of aflibercept to fluorouracil, leucovorin, and irinotecan improves survival in a phase III randomized trial in patients with metastatic colorectal cancer previously treated with an oxaliplatin-based regimen. J Clin Oncol 30 (28): 3499-506, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22949147" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 22949147</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_13">Grothey A, Van Cutsem E, Sobrero A, et al.: Regorafenib monotherapy for previously treated metastatic colorectal cancer (CORRECT): an international, multicentre, randomised, placebo-controlled, phase 3 trial. Lancet 381 (9863): 303-12, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23177514" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 23177514</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_1168_14">Kaplan MA, Isikdogan A, Gumus M, et al.: Childhood, adolescents, and young adults (&#x02264;25 y) colorectal cancer: study of Anatolian Society of Medical Oncology. J Pediatr Hematol Oncol 35 (2): 83-9, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23337551" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 23337551</span></a>]</div></li></ol></div></div><div id="CDR0000799717__1950"><h2 id="_CDR0000799717__1950_">Treatment Options Under Clinical Evaluation for Childhood Colorectal Cancer</h2><p id="CDR0000799717__1951">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, refer to 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="CDR0000799717__1952">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000799717__1953"><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 using a next-generation sequencing targeted assay of more than 4,000 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://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="CDR0000799717__722"><h2 id="_CDR0000799717__722_">Genetic Syndromes Associated With Colorectal Cancer</h2><p id="CDR0000799717__723">About 20% to 30% of adult patients with colorectal cancer have a significant history of familial cancer; of these, about 5% have a well-defined genetic syndrome.[<a class="bk_pop" href="#CDR0000799717_rl_722_1">1</a>] Hereditary colorectal cancer has two well-described forms:[<a class="bk_pop" href="#CDR0000799717_rl_722_2">2</a>,<a class="bk_pop" href="#CDR0000799717_rl_722_3">3</a>]</p><ul id="CDR0000799717__2331"><li class="half_rhythm"><div> Polyposis (including familial adenomatous polyposis [FAP] and attenuated FAP, which are caused by pathogenic variants in the <i>APC</i> gene; and <i>MUTYH</i>-associated polyposis, which is caused by pathogenic variants in the <i>MUTYH</i> gene).</div></li><li class="half_rhythm"><div>Lynch syndrome (often referred to as hereditary nonpolyposis colorectal cancer), which is caused by germline pathogenic variants in DNA mismatch repair genes (<i>MLH1</i>, <i>MSH2</i>, <i>MSH6</i>, and <i>PMS2</i>) and <i>EPCAM</i>. </div></li></ul><p id="CDR0000799717__2332">Other colorectal cancer syndromes and their associated genes include oligopolyposis (<i>POLE</i>, <i>POLD1</i>),[<a class="bk_pop" href="#CDR0000799717_rl_722_3">3</a>] <i>NTHL1</i>,[<a class="bk_pop" href="#CDR0000799717_rl_722_4">4</a>] juvenile polyposis syndrome (<i>BMPR1A</i>, <i>SMAD4</i>), Cowden syndrome (<i>PTEN</i>), and Peutz-Jeghers syndrome (<i>STK11</i>).[<a class="bk_pop" href="#CDR0000799717_rl_722_2">2</a>]</p><p id="CDR0000799717__2183">The incidence of these genetic syndromes in children has not been well defined, as follows:</p><ul id="CDR0000799717__1516"><li class="half_rhythm"><div>In one review, 16% of patients younger than 40 years had a predisposing factor for the development of colorectal cancer.[<a class="bk_pop" href="#CDR0000799717_rl_722_5">5</a>] </div></li><li class="half_rhythm"><div>A later study documented immunohistochemical evidence of mismatch repair deficiency in 31% of colorectal carcinoma samples in patients aged 30 years or younger.[<a class="bk_pop" href="#CDR0000799717_rl_722_6">6</a>]</div></li><li class="half_rhythm"><div>A retrospective review of patients younger than 18 years in Germany identified 31 patients with colorectal carcinoma.[<a class="bk_pop" href="#CDR0000799717_rl_722_7">7</a>] Eleven of the 26 patients who were tested for a genetic predisposition syndrome tested positive (eight cases of Lynch syndrome, one patient with familial adenomatous polyposis, and two patients with constitutional mismatch repair deficiency). When compared with the patients without a genetic predisposition syndrome, the 11 patients with a genetic predisposition syndrome presented with more localized disease, allowing complete surgical resection and improved outcome (100% survival).</div></li></ul><p id="CDR0000799717__724">Familial polyposis is inherited as a dominant trait, which confers a high
degree of risk. Early diagnosis and surgical removal of the colon eliminates
the risk of developing carcinomas of the large bowel.[<a class="bk_pop" href="#CDR0000799717_rl_722_8">8</a>] Some colorectal
carcinomas in young people, however, may be associated with a mutation of the
<i>APC</i> gene, which also is associated with an
increased risk of brain tumors and hepatoblastoma.[<a class="bk_pop" href="#CDR0000799717_rl_722_9">9</a>] FAP
syndrome is caused by mutation of a gene on chromosome 5q, which normally
suppresses proliferation of cells lining the intestine and later development of
polyps.[<a class="bk_pop" href="#CDR0000799717_rl_722_10">10</a>] A double-blind, placebo-controlled, randomized phase I trial in children aged 10 to 14 years with FAP reported that celecoxib at a dose of 16 mg/kg per day is safe for administration for up to 3 months. At this dose, there was a significant decrease in the number of polyps detected on colonoscopy.[<a class="bk_pop" href="#CDR0000799717_rl_722_11">11</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000587983/" class="def">Level of evidence: 1iiDiv</a>] The role of celecoxib in the management of FAP in children is not clear.</p><p id="CDR0000799717__725">Another tumor suppressor gene on chromosome 18 is associated with
progression of polyps to malignant form. Multiple colon
carcinomas have been associated with neurofibromatosis type I and
several other rare syndromes.[<a class="bk_pop" href="#CDR0000799717_rl_722_12">12</a>]</p><p id="CDR0000799717__1632">Despite the increased risk of multiple malignancies in families with Lynch syndrome, the risk of malignant neoplasms during childhood in those families does not seem to be increased when compared with the risk in children from non-Lynch syndrome colorectal carcinoma families.[<a class="bk_pop" href="#CDR0000799717_rl_722_13">13</a>]</p><div id="CDR0000799717_rl_722"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799717_rl_722_1">Gatalica Z, Torlakovic E: Pathology of the hereditary colorectal carcinoma. Fam Cancer 7 (1): 15-26, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/17564815" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 17564815</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_2">Hampel H: Genetic testing for hereditary colorectal cancer. Surg Oncol Clin N Am 18 (4): 687-703, 2009. [<a href="/pmc/articles/PMC2821160/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC2821160</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/19793575" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 19793575</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_3">Briggs S, Tomlinson I: Germline and somatic polymerase &#x003b5; and &#x003b4; mutations define a new class of hypermutated colorectal and endometrial cancers. J Pathol 230 (2): 148-53, 2013. [<a href="/pmc/articles/PMC3709119/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC3709119</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/23447401" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 23447401</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_4">Broderick P, Dobbins SE, Chubb D, et al.: Validation of Recently Proposed Colorectal Cancer Susceptibility Gene Variants in an Analysis of Families and Patients-a Systematic Review. Gastroenterology 152 (1): 75-77.e4, 2017. [<a href="/pmc/articles/PMC5860724/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC5860724</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/27713038" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 27713038</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_5">O'Connell JB, Maggard MA, Livingston EH, et al.: Colorectal cancer in the young. Am J Surg 187 (3): 343-8, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15006562" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 15006562</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_6">Goel A, Nagasaka T, Spiegel J, et al.: Low frequency of Lynch syndrome among young patients with non-familial colorectal cancer. Clin Gastroenterol Hepatol 8 (11): 966-71, 2010. [<a href="/pmc/articles/PMC2964436/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC2964436</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20655395" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 20655395</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_7">Weber ML, Schneider DT, Offenm&#x000fc;ller S, et al.: Pediatric Colorectal Carcinoma is Associated With Excellent Outcome in the Context of Cancer Predisposition Syndromes. Pediatr Blood Cancer 63 (4): 611-7, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26575411" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 26575411</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_8">Erdman SH: Pediatric adenomatous polyposis syndromes: an update. Curr Gastroenterol Rep 9 (3): 237-44, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17511923" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 17511923</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_9">Turcot J, Despres JP, St Pierre F: Malignant tumors of the central nervous system associated with familial polyposis of the colon: report of two cases. Dis Colon Rectum 2: 465-8, 1959 Sep-Oct. [<a href="https://pubmed.ncbi.nlm.nih.gov/13839882" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 13839882</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_10">Vogelstein B, Fearon ER, Hamilton SR, et al.: Genetic alterations during colorectal-tumor development. N Engl J Med 319 (9): 525-32, 1988. [<a href="https://pubmed.ncbi.nlm.nih.gov/2841597" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 2841597</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_11">Lynch PM, Ayers GD, Hawk E, et al.: The safety and efficacy of celecoxib in children with familial adenomatous polyposis. Am J Gastroenterol 105 (6): 1437-43, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20234350" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 20234350</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_12">Pratt CB, Jane JA: Multiple colorectal carcinomas, polyposis coli, and neurofibromatosis, followed by multiple glioblastoma multiforme. J Natl Cancer Inst 83 (12): 880-1, 1991. [<a href="https://pubmed.ncbi.nlm.nih.gov/1648143" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 1648143</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_rl_722_13">Heath JA, Reece JC, Buchanan DD, et al.: Childhood cancers in families with and without Lynch syndrome. Fam Cancer 14 (4): 545-51, 2015. [<a href="/pmc/articles/PMC4631656/" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pmc">PMC free article<span class="bk_prnt">: PMC4631656</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/25963852" ref="pagearea=cite-ref&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 25963852</span></a>]</div></li></ol></div></div><div id="CDR0000799717__1864"><h2 id="_CDR0000799717__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000799717__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="#CDR0000799717_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
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="CDR0000799717__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="CDR0000799717__1867"> (Refer to the PDQ <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> summaries for specific information about supportive care for children and adolescents with cancer.)</p><p id="CDR0000799717__1868">Guidelines for
pediatric cancer centers and their role in the treatment of pediatric patients
with cancer have been outlined by the American Academy of Pediatrics.[<a class="bk_pop" href="#CDR0000799717_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 in these trials 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 therapy that is currently
accepted as standard. 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="CDR0000799717__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="#CDR0000799717_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="CDR0000799717__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="#CDR0000799717_rl_1864_4">4</a>] The U.S. <a href="https://history.nih.gov/research/downloads/PL107-280.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 persons. Therefore, all pediatric cancers are considered rare.</p><p id="CDR0000799717__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="#CDR0000799717_rl_1864_5">5</a>,<a class="bk_pop" href="#CDR0000799717_rl_1864_6">6</a>] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000799717__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="#CDR0000799717_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="#CDR0000799717_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="#CDR0000799717_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="CDR0000799717__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="CDR0000799717__1829">Information about these tumors may also be found in sources relevant to
adults with cancer such as the PDQ summaries on adult <a href="/books/n/pdqcis/CDR0000062687/">Colon
Cancer Treatment</a> and adult <a href="/books/n/pdqcis/CDR0000062726/">Rectal Cancer Treatment</a>.</p><div id="CDR0000799717_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799717_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="CDR0000799717_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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 15173520</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_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="CDR0000799717_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&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="CDR0000799717_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&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="CDR0000799717_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&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="CDR0000799717_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&amp;targetsite=entrez&amp;targetcat=link&amp;targettype=pubmed">PubMed<span class="bk_prnt">: 17049226</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799717_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&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><li><div class="bk_ref" id="CDR0000799717_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&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">Also available online</a>. Last accessed February 20, 2020.</div></li></ol></div></div><div id="CDR0000799717__2353"><h2 id="_CDR0000799717__2353_">Changes to This Summary (10/22/2019)</h2><p id="CDR0000799717__2354">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="CDR0000799717__2355">This is a new summary.</p><p id="CDR0000799717__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="#CDR0000799717__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; - NCI's Comprehensive Cancer Database</a> pages.
</p></div><div id="CDR0000799717__AboutThis_1"><h2 id="_CDR0000799717__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000799717__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000799717__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric colorectal 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="CDR0000799717__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000799717__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="CDR0000799717__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000799717__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="CDR0000799717__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 Colorectal 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, FACR (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>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="CDR0000799717__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="CDR0000799717__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000799717__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="CDR0000799717__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000799717__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="CDR0000799717__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000799717__AboutThis_15">PDQ&#x000ae; Pediatric Treatment Editorial Board. PDQ Childhood Colorectal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated &#x0003c;MM/DD/YYYY&#x0003e;. Available at: <a href="https://www.cancer.gov/types/colorectal/hp/child-colorectal-treatment-pdq" ref="pagearea=body&amp;targetsite=external&amp;targetcat=link&amp;targettype=uri">https://www.cancer.gov/types/colorectal/hp/child-colorectal-treatment-pdq</a>. Accessed &#x0003c;MM/DD/YYYY&#x0003e;. </p><p id="CDR0000799717__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="CDR0000799717__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000799717__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="CDR0000799717__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000799717__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>
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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/NBK549125.1/?report=reader">PubReader</a></li><li><a href="/books/NBK549125.1/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK549125" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK549125" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Pediatric Treatment Editorial Board. 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ref="log$=inpage&amp;link_id=inpage">Staging</a></li><li><a href="#CDR0000799717__1168" ref="log$=inpage&amp;link_id=inpage">Treatment and Outcome of Childhood Colorectal Cancer</a></li><li><a href="#CDR0000799717__1950" ref="log$=inpage&amp;link_id=inpage">Treatment Options Under Clinical Evaluation for Childhood Colorectal Cancer</a></li><li><a href="#CDR0000799717__722" ref="log$=inpage&amp;link_id=inpage">Genetic Syndromes Associated With Colorectal Cancer</a></li><li><a href="#CDR0000799717__1864" ref="log$=inpage&amp;link_id=inpage">Special Considerations for the Treatment of Children With Cancer</a></li><li><a href="#CDR0000799717__2353" ref="log$=inpage&amp;link_id=inpage">Changes to This Summary (10/22/2019)</a></li><li><a href="#CDR0000799717__AboutThis_1" ref="log$=inpage&amp;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 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