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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="#__NBK475848_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK475848_dtls__"><div>Bethesda (MD): <a href="http://www.cancer.gov/" ref="pagearea=page-banner&targetsite=external&targetcat=link&targettype=publisher">National Cancer Institute (US)</a>; 2002-.</div></div><div class="half_rhythm"></div><div class="bk_noprnt"><form method="get" action="/books/n/pdqcis/" id="bk_srch"><div class="bk_search"><label for="bk_term" class="offscreen_noflow">Search term</label><input type="text" title="Search this book" id="bk_term" name="term" value="" data-jig="ncbiclearbutton" /> <input type="submit" class="jig-ncbibutton" value="Search this book" submit="false" style="padding: 0.1em 0.4em;" /></div></form></div></div></div></div></div>
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<div class="main-content lit-style" itemscope="itemscope" itemtype="http://schema.org/CreativeWork"><div class="meta-content fm-sec"><h1 id="_NBK475848_"><span class="title" itemprop="name">Childhood Esthesioneuroblastoma Treatment (PDQ®)</span></h1><div class="subtitle whole_rhythm">Health Professional Version</div><p class="contrib-group"><span itemprop="author">PDQ Pediatric Treatment Editorial Board</span>.</p><p class="small">Published online: January 8, 2018.</p><p class="small">Created: <span itemprop="datePublished">January 8, 2018</span>.</p></div><div class="jig-ncbiinpagenav body-content whole_rhythm" data-jigconfig="allHeadingLevels: ['h2'],smoothScroll: false" itemprop="text"><div id="_abs_rndgid_" itemprop="description"><p id="CDR0000790381__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood esthesioneuroblastoma. 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="CDR0000790381__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="CDR0000790381__1386"><h2 id="_CDR0000790381__1386_">Incidence</h2><p id="CDR0000790381__117">Esthesioneuroblastoma (olfactory neuroblastoma) is a small round-cell tumor arising from the nasal neuroepithelium that is distinct from primitive neuroectodermal tumors.[<a class="bk_pop" href="#CDR0000790381_rl_1386_1">1</a>-<a class="bk_pop" href="#CDR0000790381_rl_1386_4">4</a>] In children, esthesioneuroblastoma is a very rare malignancy, with an estimated incidence of 0.1 cases per 100,000 per year in children younger than 15 years.[<a class="bk_pop" href="#CDR0000790381_rl_1386_5">5</a>]</p><p id="CDR0000790381__1244">Despite its rarity, esthesioneuroblastoma is the most common cancer of the nasal cavity in pediatric patients, accounting for 28% of cases in a Surveillance, Epidemiology, and End Results (SEER) study.[<a class="bk_pop" href="#CDR0000790381_rl_1386_6">6</a>] In a series of 511 patients from the SEER database, there was a slight male predominance, the mean age at presentation was 53 years, and only 8% of cases were younger than 25 years.[<a class="bk_pop" href="#CDR0000790381_rl_1386_7">7</a>] Most patients were white (81%) and the most common tumor sites were the nasal cavity (72%) and ethmoid sinus (13%).[<a class="bk_pop" href="#CDR0000790381_rl_1386_7">7</a>]</p><div id="CDR0000790381_rl_1386"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000790381_rl_1386_1">Kumar M, Fallon RJ, Hill JS, et al.: Esthesioneuroblastoma in children. J Pediatr Hematol Oncol 24 (6): 482-7, 2002 Aug-Sep. [<a href="https://pubmed.ncbi.nlm.nih.gov/12218598" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12218598</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1386_2">Theilgaard SA, Buchwald C, Ingeholm P, et al.: Esthesioneuroblastoma: a Danish demographic study of 40 patients registered between 1978 and 2000. Acta Otolaryngol 123 (3): 433-9, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/12737303" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12737303</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1386_3">Dias FL, Sa GM, Lima RA, et al.: Patterns of failure and outcome in esthesioneuroblastoma. Arch Otolaryngol Head Neck Surg 129 (11): 1186-92, 2003. [<a href="https://pubmed.ncbi.nlm.nih.gov/14623748" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 14623748</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1386_4">Nakao K, Watanabe K, Fujishiro Y, et al.: Olfactory neuroblastoma: long-term clinical outcome at a single institute between 1979 and 2003. Acta Otolaryngol Suppl (559): 113-7, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/18340581" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18340581</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1386_5">Bisogno G, Soloni P, Conte M, et al.: Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees. BMC Cancer 12: 117, 2012. [<a href="/pmc/articles/PMC3368746/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3368746</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/22443159" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22443159</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1386_6">Benoit MM, Bhattacharyya N, Faquin W, et al.: Cancer of the nasal cavity in the pediatric population. Pediatrics 121 (1): e141-5, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18086819" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18086819</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1386_7">Soler ZM, Smith TL: Endoscopic versus open craniofacial resection of esthesioneuroblastoma: what is the evidence? Laryngoscope 122 (2): 244-5, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22261850" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22261850</span></a>]</div></li></ol></div></div><div id="CDR0000790381__1387"><h2 id="_CDR0000790381__1387_">Clinical Presentation</h2><p id="CDR0000790381__651">Most children present in the second decade of life with symptoms that include the following: </p><ul id="CDR0000790381__1245"><li class="half_rhythm"><div>Nasal obstruction.</div></li><li class="half_rhythm"><div>Epistaxis.</div></li><li class="half_rhythm"><div>Hyposmia.</div></li><li class="half_rhythm"><div>Exophthalmos.</div></li><li class="half_rhythm"><div>Nasopharyngeal mass, which may have local extension into the orbits, sinuses, or frontal lobe.</div></li></ul></div><div id="CDR0000790381__1837"><h2 id="_CDR0000790381__1837_">Prognostic Factors</h2><p id="CDR0000790381__1838">Review of multiple case series of mainly adult patients indicate that the following may correlate with adverse prognosis:[<a class="bk_pop" href="#CDR0000790381_rl_1837_1">1</a>-<a class="bk_pop" href="#CDR0000790381_rl_1837_3">3</a>]</p><ul id="CDR0000790381__1839"><li class="half_rhythm"><div>Higher histopathologic grade. </div></li><li class="half_rhythm"><div>Positive surgical margin status.</div></li><li class="half_rhythm"><div>Metastases to the cervical lymph nodes.</div></li></ul><div id="CDR0000790381_rl_1837"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000790381_rl_1837_1">Dulguerov P, Allal AS, Calcaterra TC: Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2 (11): 683-90, 2001. [<a href="https://pubmed.ncbi.nlm.nih.gov/11902539" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 11902539</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1837_2">Patel SG, Singh B, Stambuk HE, et al.: Craniofacial surgery for esthesioneuroblastoma: report of an international collaborative study. J Neurol Surg B Skull Base 73 (3): 208-20, 2012. [<a href="/pmc/articles/PMC3424016/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3424016</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/23730550" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23730550</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1837_3">Herr MW, Sethi RK, Meier JC, et al.: Esthesioneuroblastoma: an update on the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy. J Neurol Surg B Skull Base 75 (1): 58-64, 2014. [<a href="/pmc/articles/PMC3912136/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3912136</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/24498591" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24498591</span></a>]</div></li></ol></div></div><div id="CDR0000790381__1388"><h2 id="_CDR0000790381__1388_">Stage Information for Childhood Esthesioneuroblastoma</h2><p id="CDR0000790381__1223">Tumors are staged according to the Kadish system (refer to <a class="figpopup" href="/books/NBK475848.1/table/CDR0000790381__1231/?report=objectonly" target="object" rid-figpopup="figCDR00007903811231" rid-ob="figobCDR00007903811231">Table 1</a>). Correlated with Kadish stage, survival ranges from 90% (stage A) to less than 40% (stage D). Most patients present with locally advanced–stage disease (Kadish stages B and C) and almost one-third of patients have tumors at distant sites (Kadish stage D).[<a class="bk_pop" href="#CDR0000790381_rl_1388_1">1</a>,<a class="bk_pop" href="#CDR0000790381_rl_1388_2">2</a>] Recent reports suggest that positron emission tomography–computed tomography (PET-CT) may aid in staging the disease.[<a class="bk_pop" href="#CDR0000790381_rl_1388_3">3</a>]</p><div id="CDR0000790381__1231" class="table"><h3><span class="title">Table 1. Kadish Staging System</span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK475848.1/table/CDR0000790381__1231/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000790381__1231_lrgtbl__"><table class="no_top_margin"><thead><tr><th colspan="1" rowspan="1" style="vertical-align:top;">Stage</th><th colspan="1" rowspan="1" style="vertical-align:top;">Description</th></tr></thead><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;"> A</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor confined to the nasal cavity.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">B</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor extending to the nasal sinuses.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">C</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor extending to the nasal sinuses and beyond.</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">D</td><td colspan="1" rowspan="1" style="vertical-align:top;">Tumor metastases present.</td></tr></tbody></table></div></div><div id="CDR0000790381_rl_1388"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000790381_rl_1388_1">Bisogno G, Soloni P, Conte M, et al.: Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees. BMC Cancer 12: 117, 2012. [<a href="/pmc/articles/PMC3368746/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3368746</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/22443159" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22443159</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1388_2">Benoit MM, Bhattacharyya N, Faquin W, et al.: Cancer of the nasal cavity in the pediatric population. Pediatrics 121 (1): e141-5, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18086819" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18086819</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1388_3">Broski SM, Hunt CH, Johnson GB, et al.: The added value of 18F-FDG PET/CT for evaluation of patients with esthesioneuroblastoma. J Nucl Med 53 (8): 1200-6, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22728262" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22728262</span></a>]</div></li></ol></div></div><div id="CDR0000790381__1246"><h2 id="_CDR0000790381__1246_">Treatment and Outcome of Childhood Esthesioneuroblastoma</h2><p id="CDR0000790381__652">The use of multimodal therapy optimizes the chances for survival, with more than 70% of children expected to survive 5 or more years after initial diagnosis.[<a class="bk_pop" href="#CDR0000790381_rl_1246_1">1</a>-<a class="bk_pop" href="#CDR0000790381_rl_1246_3">3</a>] A multi-institutional review of 24 patients younger than 21 years at diagnosis found a 5-year disease-free survival and overall survival of 73% to 74%.[<a class="bk_pop" href="#CDR0000790381_rl_1246_4">4</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335150/" class="def">Level of evidence: 3iiiA</a>]</p><p id="CDR0000790381__1324">Treatment options according to Kadish stage include the following:[<a class="bk_pop" href="#CDR0000790381_rl_1246_5">5</a>]</p><ol id="CDR0000790381__1456"><li class="half_rhythm"><div><b>Kadish stage A:</b>
|
|
Surgery alone with clear margins. Adjuvant radiation therapy is indicated in patients with close and positive margins or with residual disease.</div></li><li class="half_rhythm"><div><b>Kadish stage B:</b>
|
|
Surgery followed by adjuvant radiation therapy. The role of adjuvant chemotherapy is controversial.</div></li><li class="half_rhythm"><div><b>Kadish stage C:</b>
|
|
Neoadjuvant approach with chemotherapy, radiation therapy, or concurrent chemotherapy-radiation therapy followed by surgery.</div></li><li class="half_rhythm"><div><b>Kadish stage D:</b>
|
|
Systemic chemotherapy and palliative radiation therapy to local and metastatic sites. Palliative care is incorporated into the treatment plan to improve quality of life.</div></li></ol><p id="CDR0000790381__569">The mainstay of treatment is surgery and radiation.[<a class="bk_pop" href="#CDR0000790381_rl_1246_6">6</a>] Newer techniques such as endoscopic sinus surgery may offer similar short-term outcomes to open craniofacial resection.[<a class="bk_pop" href="#CDR0000790381_rl_1246_7">7</a>]; [<a class="bk_pop" href="#CDR0000790381_rl_1246_8">8</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335155/" class="def">Level of evidence: 3iiiDii</a>] Other techniques such as <a href="/books/n/pdqcis/glossary/def-item/glossary_CDR0000046701/" class="def">stereotactic radiosurgery</a> and proton-beam therapy (<a href="/books/n/pdqcis/glossary/def-item/glossary_CDR0000534234/" class="def">charged-particle radiation therapy</a>) may also play a role in the management of this tumor.[<a class="bk_pop" href="#CDR0000790381_rl_1246_3">3</a>,<a class="bk_pop" href="#CDR0000790381_rl_1246_9">9</a>]</p><p id="CDR0000790381__1247">Nodal metastases are seen in about 5% of patients. Routine neck dissection and nodal exploration are not indicated in the absence of clinical or radiological evidence of disease.[<a class="bk_pop" href="#CDR0000790381_rl_1246_10">10</a>] Management of cervical lymph node metastases has been addressed in a review article.[<a class="bk_pop" href="#CDR0000790381_rl_1246_10">10</a>] </p><p id="CDR0000790381__570">Reports indicate promising results with the increased use of resection and neoadjuvant or adjuvant chemotherapy in patients with advanced-stage disease.[<a class="bk_pop" href="#CDR0000790381_rl_1246_2">2</a>,<a class="bk_pop" href="#CDR0000790381_rl_1246_4">4</a>,<a class="bk_pop" href="#CDR0000790381_rl_1246_11">11</a>-<a class="bk_pop" href="#CDR0000790381_rl_1246_13">13</a>]; [<a class="bk_pop" href="#CDR0000790381_rl_1246_14">14</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000561227/" class="def">Level of evidence: 3iii</a>] Chemotherapy regimens that have been used with efficacy include cisplatin and etoposide with or without ifosfamide;[<a class="bk_pop" href="#CDR0000790381_rl_1246_5">5</a>,<a class="bk_pop" href="#CDR0000790381_rl_1246_15">15</a>] vincristine, actinomycin D, and cyclophosphamide with or without doxorubicin; ifosfamide and etoposide; cisplatin plus etoposide or doxorubicin;[<a class="bk_pop" href="#CDR0000790381_rl_1246_2">2</a>] vincristine, doxorubicin, and cyclophosphamide;[<a class="bk_pop" href="#CDR0000790381_rl_1246_16">16</a>] and irinotecan plus docetaxel.[<a class="bk_pop" href="#CDR0000790381_rl_1246_17">17</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335144/" class="def">Level of evidence: 3iiA</a>] </p><div id="CDR0000790381_rl_1246"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000790381_rl_1246_1">Bisogno G, Soloni P, Conte M, et al.: Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees. BMC Cancer 12: 117, 2012. [<a href="/pmc/articles/PMC3368746/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3368746</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/22443159" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22443159</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_2">Eich HT, Müller RP, Micke O, et al.: Esthesioneuroblastoma in childhood and adolescence. Better prognosis with multimodal treatment? Strahlenther Onkol 181 (6): 378-84, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15925980" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15925980</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_3">Lucas JT Jr, Ladra MM, MacDonald SM, et al.: Proton therapy for pediatric and adolescent esthesioneuroblastoma. Pediatr Blood Cancer 62 (9): 1523-8, 2015. [<a href="/pmc/articles/PMC9124545/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC9124545</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/25820437" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25820437</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_4">Venkatramani R, Pan H, Furman WL, et al.: Multimodality Treatment of Pediatric Esthesioneuroblastoma. Pediatr Blood Cancer 63 (3): 465-70, 2016. [<a href="/pmc/articles/PMC5134836/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5134836</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/26514449" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26514449</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_5">Kumar R: Esthesioneuroblastoma: Multimodal management and review of literature. World J Clin Cases 3 (9): 774-8, 2015. [<a href="/pmc/articles/PMC4568526/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4568526</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/26380824" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26380824</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_6">Ozsahin M, Gruber G, Olszyk O, et al.: Outcome and prognostic factors in olfactory neuroblastoma: a rare cancer network study. Int J Radiat Oncol Biol Phys 78 (4): 992-7, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20231062" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20231062</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_7">Soler ZM, Smith TL: Endoscopic versus open craniofacial resection of esthesioneuroblastoma: what is the evidence? Laryngoscope 122 (2): 244-5, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22261850" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22261850</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_8">Gallia GL, Reh DD, Lane AP, et al.: Endoscopic resection of esthesioneuroblastoma. J Clin Neurosci 19 (11): 1478-82, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22995757" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22995757</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_9">Unger F, Haselsberger K, Walch C, et al.: Combined endoscopic surgery and radiosurgery as treatment modality for olfactory neuroblastoma (esthesioneuroblastoma). Acta Neurochir (Wien) 147 (6): 595-601; discussion 601-2, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15806328" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15806328</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_10">Zanation AM, Ferlito A, Rinaldo A, et al.: When, how and why to treat the neck in patients with esthesioneuroblastoma: a review. Eur Arch Otorhinolaryngol 267 (11): 1667-71, 2010. [<a href="/pmc/articles/PMC3005584/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3005584</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20706843" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20706843</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_11">Kumar M, Fallon RJ, Hill JS, et al.: Esthesioneuroblastoma in children. J Pediatr Hematol Oncol 24 (6): 482-7, 2002 Aug-Sep. [<a href="https://pubmed.ncbi.nlm.nih.gov/12218598" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12218598</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_12">Loy AH, Reibel JF, Read PW, et al.: Esthesioneuroblastoma: continued follow-up of a single institution's experience. Arch Otolaryngol Head Neck Surg 132 (2): 134-8, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16490869" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16490869</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_13">Porter AB, Bernold DM, Giannini C, et al.: Retrospective review of adjuvant chemotherapy for esthesioneuroblastoma. J Neurooncol 90 (2): 201-4, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18633576" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18633576</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_14">Benfari G, Fusconi M, Ciofalo A, et al.: Radiotherapy alone for local tumour control in esthesioneuroblastoma. Acta Otorhinolaryngol Ital 28 (6): 292-7, 2008. [<a href="/pmc/articles/PMC2689543/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2689543</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/19205593" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19205593</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_15">Kim DW, Jo YH, Kim JH, et al.: Neoadjuvant etoposide, ifosfamide, and cisplatin for the treatment of olfactory neuroblastoma. Cancer 101 (10): 2257-60, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15484215" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15484215</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_16">El Kababri M, Habrand JL, Valteau-Couanet D, et al.: Esthesioneuroblastoma in children and adolescent: experience on 11 cases with literature review. J Pediatr Hematol Oncol 36 (2): 91-5, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/24390450" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24390450</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1246_17">Kiyota N, Tahara M, Fujii S, et al.: Nonplatinum-based chemotherapy with irinotecan plus docetaxel for advanced or metastatic olfactory neuroblastoma: a retrospective analysis of 12 cases. Cancer 112 (4): 885-91, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18189294" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18189294</span></a>]</div></li></ol></div></div><div id="CDR0000790381__1796"><h2 id="_CDR0000790381__1796_">Treatment Options Under Clinical Evaluation for Childhood Esthesioneuroblastoma</h2><p id="CDR0000790381__1873">Information about National Cancer Institute (NCI)–supported clinical trials can be found on the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/search" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>. For information about clinical trials sponsored by other organizations, refer to the <a href="https://clinicaltrials.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a>.</p><p id="CDR0000790381__1797">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000790381__1798"><li class="half_rhythm"><div class="half_rhythm"><b><a href="http://cancer.gov/clinicaltrials/search/view?version=healthprofessional&cdrid=789765" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">APEC1621 (NCT03155620)</a></b> (Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients with Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders)<b>:</b> NCI-COG Pediatric Molecular Analysis for Therapeutic Choice (MATCH), referred to as Pediatric MATCH, will match targeted agents with specific molecular changes identified using a next-generation sequencing targeted assay of more than 3,000 different mutations across more than 160 genes in refractory and recurrent solid tumors. Children and adolescents aged 1 to 21 years are eligible for the trial.</div><div class="half_rhythm">Tumor tissue from progressive or recurrent disease must be available for molecular characterization. Patients with tumors that have molecular variants addressed by treatment arms included in the trial will be offered treatment on Pediatric MATCH. Additional information can be obtained on the <a href="https://clinicaltrials.gov" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a> for <a href="https://clinicaltrials.gov/ct2/show/NCT03155620" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">APEC1621 (NCT03155620)</a>.
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</div></li></ul></div><div id="CDR0000790381__1864"><h2 id="_CDR0000790381__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000790381__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="#CDR0000790381_rl_1864_1">1</a>] Referral to medical centers with multidisciplinary teams of cancer specialists experienced in treating cancers that occur in childhood and adolescence should be considered for children and adolescents with cancer. This multidisciplinary team approach incorporates the skills
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of the following health care professionals and others to ensure that children receive treatment, supportive care, and rehabilitation
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that will achieve optimal survival and quality of life:</p><ul id="CDR0000790381__1866"><li class="half_rhythm"><div>Primary care physicians.</div></li><li class="half_rhythm"><div>Pediatric surgeons.</div></li><li class="half_rhythm"><div>Radiation
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oncologists.</div></li><li class="half_rhythm"><div>Pediatric medical oncologists/hematologists.</div></li><li class="half_rhythm"><div> Rehabilitation
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specialists.</div></li><li class="half_rhythm"><div>Pediatric nurse specialists.</div></li><li class="half_rhythm"><div>Social workers.</div></li><li class="half_rhythm"><div>Child-life professionals.</div></li><li class="half_rhythm"><div>Psychologists.</div></li></ul><p id="CDR0000790381__1867"> (Refer to the PDQ <a href="https://www.cancer.gov/publications/pdq/information-summaries/supportive-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Supportive and Palliative Care</a> summaries for specific information about supportive care for children and adolescents with cancer.)</p><p id="CDR0000790381__1868">Guidelines for
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pediatric cancer centers and their role in the treatment of pediatric patients
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with cancer have been outlined by the American Academy of Pediatrics.[<a class="bk_pop" href="#CDR0000790381_rl_1864_2">2</a>] At
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these pediatric cancer centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity
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to participate in these trials is offered to most patients and their families. Clinical
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trials for children and adolescents diagnosed with cancer are generally
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designed to compare potentially better therapy with therapy that is currently
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accepted as standard. Most of the progress made in identifying curative
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therapy for childhood cancers has been achieved through clinical trials.
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Information about ongoing clinical trials is available from the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>.</p><p id="CDR0000790381__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="#CDR0000790381_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="CDR0000790381__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="#CDR0000790381_rl_1864_4">4</a>] The U.S. <a href="https://history.nih.gov/research/downloads/PL107-280.pdf" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Rare Diseases Act of 2002</a> defines a rare disease as one that affects populations smaller than 200,000 persons and, by definition, all pediatric cancers are considered rare. The designation of a pediatric rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000790381__1871"><li class="half_rhythm"><div>The Italian Rare Tumors in Pediatric Age (TREP) cooperative project 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="#CDR0000790381_rl_1864_5">5</a>]</div></li><li class="half_rhythm"><div>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="#CDR0000790381_rl_1864_6">6</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="#CDR0000790381_rl_1864_7">7</a>] 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="CDR0000790381__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><div id="CDR0000790381_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000790381_rl_1864_1">Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010. [<a href="/pmc/articles/PMC2881732/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2881732</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20404250" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20404250</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1864_2">Corrigan JJ, Feig SA; American Academy of Pediatrics: Guidelines for pediatric cancer centers. Pediatrics 113 (6): 1833-5, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15173520" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15173520</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1864_3">Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014. [<a href="/pmc/articles/PMC4136455/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4136455</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/24853691" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24853691</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1864_4">Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr. [<a href="https://pubmed.ncbi.nlm.nih.gov/24488779" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24488779</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1864_5">Ferrari A, Bisogno G, De Salvo GL, et al.: The challenge of very rare tumours in childhood: the Italian TREP project. Eur J Cancer 43 (4): 654-9, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17049226" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17049226</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1864_6">Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010. [<a href="/pmc/articles/PMC3020699/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3020699</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20956621" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20956621</span></a>]</div></li><li><div class="bk_ref" id="CDR0000790381_rl_1864_7">Howlader N, Noone AM, Krapcho M, et al., eds.: SEER Cancer Statistics Review, 1975-2012. Bethesda, Md: National Cancer Institute, 2015. <a href="http://seer.cancer.gov/csr/1975_2012/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Also available online</a>. Last accessed August 13, 2018.</div></li></ol></div></div><div id="CDR0000790381__1840"><h2 id="_CDR0000790381__1840_">Changes to This Summary (01/08/2018)</h2><p id="CDR0000790381__1841">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="CDR0000790381__1842">This is a new summary.</p><p id="CDR0000790381__disclaimerHP_3">This summary is written and maintained by the <a href="http://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric Treatment Editorial Board</a>, which is
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editorially independent of NCI. The summary reflects an independent review of
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the literature and does not represent a policy statement of NCI or NIH. More
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information about summary policies and the role of the PDQ Editorial Boards in
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maintaining the PDQ summaries can be found on the <a href="#CDR0000790381__AboutThis_1">About This PDQ Summary</a> and <a href="http://www.cancer.gov/publications/pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ® - NCI's Comprehensive Cancer Database</a> pages.
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</p></div><div id="CDR0000790381__AboutThis_1"><h2 id="_CDR0000790381__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000790381__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000790381__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood esthesioneuroblastoma. 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="CDR0000790381__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000790381__AboutThis_5">This summary is reviewed regularly and updated as necessary by the <a href="http://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric Treatment Editorial Board</a>, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).</p><p id="CDR0000790381__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000790381__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="CDR0000790381__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 Esthesioneuroblastoma Treatment are:</p><ul><li class="half_rhythm"><div>Denise Adams, MD (Children's Hospital Boston)</div></li><li class="half_rhythm"><div>Karen J. Marcus, MD (Dana-Farber Cancer Institute/Boston Children's Hospital)</div></li><li class="half_rhythm"><div>Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)</div></li><li class="half_rhythm"><div>Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)</div></li><li class="half_rhythm"><div>Alberto S. Pappo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>R Beverly Raney, MD (Consultant)</div></li><li class="half_rhythm"><div>Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)</div></li><li class="half_rhythm"><div>Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>Stephen J. Shochat, MD (St. Jude Children's Research Hospital)</div></li></ul><p id="CDR0000790381__AboutThis_9">Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.</p></div><div id="CDR0000790381__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000790381__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="CDR0000790381__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000790381__AboutThis_13">PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”</p><p id="CDR0000790381__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000790381__AboutThis_15">PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Esthesioneuroblastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/head-and-neck/hp/child/esthesioneuroblastoma-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/head-and-neck/hp/child/esthesioneuroblastoma-treatment-pdq</a>. Accessed <MM/DD/YYYY>. </p><p id="CDR0000790381__AboutThis_16">Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in <a href="https://visualsonline.cancer.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Visuals Online</a>, a collection of over 2,000 scientific images.
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</p></div><div id="CDR0000790381__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000790381__AboutThis_18">Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the <a href="https://www.cancer.gov/about-cancer/managing-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Managing Cancer Care</a> page.</p></div><div id="CDR0000790381__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000790381__AboutThis_21">More information about contacting us or receiving help with the Cancer.gov website can be found on our <a href="https://www.cancer.gov/contact" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Contact Us for Help</a> page. Questions can also be submitted to Cancer.gov through the website’s <a href="https://www.cancer.gov/contact/email-us" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Email Us</a>.</p></div></div></div></div>
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2018</li><li><span class="bk_col_itm"><a href="/books/NBK475848.2/">NBK475848.2</a></span> August 28, 2018</li><li><span class="bk_col_itm">NBK475848.1</span> January 8, 2018 (Displayed Version)</li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>In this Page</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="page-toc" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="#CDR0000790381__1386" ref="log$=inpage&link_id=inpage">Incidence</a></li><li><a href="#CDR0000790381__1387" ref="log$=inpage&link_id=inpage">Clinical Presentation</a></li><li><a href="#CDR0000790381__1837" ref="log$=inpage&link_id=inpage">Prognostic Factors</a></li><li><a href="#CDR0000790381__1388" ref="log$=inpage&link_id=inpage">Stage Information for Childhood Esthesioneuroblastoma</a></li><li><a href="#CDR0000790381__1246" ref="log$=inpage&link_id=inpage">Treatment and Outcome of Childhood Esthesioneuroblastoma</a></li><li><a href="#CDR0000790381__1796" ref="log$=inpage&link_id=inpage">Treatment Options Under Clinical Evaluation for Childhood Esthesioneuroblastoma</a></li><li><a href="#CDR0000790381__1864" ref="log$=inpage&link_id=inpage">Special Considerations for the Treatment of Children With Cancer</a></li><li><a href="#CDR0000790381__1840" ref="log$=inpage&link_id=inpage">Changes to This Summary (01/08/2018)</a></li><li><a href="#CDR0000790381__AboutThis_1" ref="log$=inpage&link_id=inpage">About This PDQ Summary</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related publications</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="document-links" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="/books/NBK475814/">Patient Version</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related information</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="discovery_db_links" id="Shutter"></a></div><div class="portlet_content"><ul><li class="brieflinkpopper"><a class="brieflinkpopperctrl" href="/books/?Db=pmc&DbFrom=books&Cmd=Link&LinkName=books_pmc_refs&IdsFromResult=4490060" ref="log$=recordlinks">PMC</a><div class="brieflinkpop offscreen_noflow">PubMed Central citations</div></li><li class="brieflinkpopper"><a class="brieflinkpopperctrl" href="/books/?Db=pubmed&DbFrom=books&Cmd=Link&LinkName=books_pubmed_refs&IdsFromResult=4490060" ref="log$=recordlinks">PubMed</a><div class="brieflinkpop offscreen_noflow">Links to PubMed</div></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Similar articles in PubMed</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="PBooksDiscovery_RA" id="Shutter"></a></div><div class="portlet_content"><ul><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/29337482" ref="ordinalpos=1&linkpos=1&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Oral Cavity Cancer Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Oral Cavity Cancer Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389243" ref="ordinalpos=1&linkpos=2&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/31593397" ref="ordinalpos=1&linkpos=3&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Mesothelioma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Mesothelioma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/26389330" ref="ordinalpos=1&linkpos=4&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Craniopharyngioma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Craniopharyngioma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/31909942" ref="ordinalpos=1&linkpos=5&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Pheochromocytoma and Paraganglioma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Pheochromocytoma and Paraganglioma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li></ul><a class="seemore" href="/sites/entrez?db=pubmed&cmd=link&linkname=pubmed_pubmed_reviews&uid=29337483" ref="ordinalpos=1&log$=relatedreviews_seeall&logdbfrom=pubmed">See reviews...</a><a class="seemore" href="/sites/entrez?db=pubmed&cmd=link&linkname=pubmed_pubmed&uid=29337483" ref="ordinalpos=1&log$=relatedarticles_seeall&logdbfrom=pubmed">See all...</a></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Recent Activity</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="recent_activity" id="Shutter"></a></div><div class="portlet_content"><div xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" id="HTDisplay" class=""><div class="action"><a href="javascript:historyDisplayState('ClearHT')">Clear</a><a href="javascript:historyDisplayState('HTOff')" class="HTOn">Turn Off</a><a href="javascript:historyDisplayState('HTOn')" class="HTOff">Turn On</a></div><ul id="activity"><li class="ra_rcd ralinkpopper 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