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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="#__NBK65891_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK65891_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="_NBK65891_"><span class="title" itemprop="name">Childhood Craniopharyngioma 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 26, 2021.</p><p class="small">Created: <span itemprop="datePublished">December 24, 2008</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="CDR0000589150__186">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood craniopharyngioma. 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="CDR0000589150__187">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="CDR0000589150__4"><h2 id="_CDR0000589150__4_">General Information About Childhood Craniopharyngioma</h2><p id="CDR0000589150__39">Primary brain tumors, including craniopharyngiomas, are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. </p><p id="CDR0000589150__136">Craniopharyngiomas are uncommon pediatric brain tumors. They are believed to be congenital in origin, arising from ectodermal remnants, Rathke cleft, or other embryonal epithelium, and often occur in the suprasellar region with an intrasellar portion. Magnetic resonance imaging (MRI) and computed tomography (CT) imaging are used to diagnose craniopharyngiomas, but histologic confirmation is generally required before treatment. The treatment of patients with newly diagnosed craniopharyngiomas may include a combination of surgery, radiation therapy, cyst drainage, and/or intracystic interferon-alpha. The treatment of patients with recurrent craniopharyngiomas depends on the initial treatment used. Regardless of treatment given, the 5-year and 10-year survival rates exceed 90% for children between the ages of 0 and 14 years.[<a class="bk_pop" href="#CDR0000589150_rl_4_1">1</a>]</p><p id="CDR0000589150__1798">The PDQ childhood brain tumor treatment summaries are organized primarily according to the World Health Organization classification of nervous system tumors.[<a class="bk_pop" href="#CDR0000589150_rl_4_2">2</a>] For a full description of the classification of nervous system tumors and a link to the corresponding treatment summary for each type of brain tumor, refer to the PDQ summary on <a href="/books/n/pdqcis/CDR0000062680/">Childhood Brain and Spinal Cord Tumors Treatment Overview</a>.</p><div id="CDR0000589150__40"><h3> Incidence </h3><p id="CDR0000589150__41">Craniopharyngiomas are relatively uncommon, accounting for about 6% of all intracranial tumors in children.[<a class="bk_pop" href="#CDR0000589150_rl_4_3">3</a>,<a class="bk_pop" href="#CDR0000589150_rl_4_4">4</a>] </p><p id="CDR0000589150__138">No predisposing factors have been identified.</p></div><div id="CDR0000589150__139"><h3>Anatomy</h3><a id="CDR0000589150__140"></a><div id="CDR0000589150__135" class="figure bk_fig"><div class="graphic"><a href="/core/lw/2.0/html/tileshop_pmc/tileshop_pmc_inline.html?title=Figure%201&p=BOOKS&id=567186_CDR0000748659.jpg" target="tileshopwindow" class="inline_block pmc_inline_block ts_canvas img_link" title="Click on image to zoom"><div class="ts_bar small" title="Click on image to zoom"></div><img src="/books/NBK65891.14/bin/CDR0000748659.jpg" alt="Drawing of the inside of the brain showing the supratentorial area (the upper part of the brain) and the posterior fossa/infratentorial area (the lower back part of the brain). The supratentorial area contains the cerebrum, lateral ventricle, third ventricle, choroid plexus, hypothalamus, pineal gland, pituitary gland, and optic nerve. The posterior fossa/infratentorial area contains the cerebellum, tectum, fourth ventricle, and brain stem (pons and medulla). The tentorium and spinal cord are also shown." class="tileshop" title="Click on image to zoom" /></a></div><div class="caption"><p>Figure 1. Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other parts of the brain. The tentorium separates the cerebrum from the cerebellum. The infratentorium (posterior fossa) is the region below the tentorium that contains the brain stem, cerebellum, and fourth ventricle. The supratentorium is the region above the tentorium and denotes the region that contains the cerebrum. </p></div></div></div><div id="CDR0000589150__176"><h3>Clinical Presentation</h3><p id="CDR0000589150__177">Craniopharyngiomas occur in the region of the pituitary gland, and endocrine function may be affected (refer to <a class="figpopup" href="/books/NBK65891.14/figure/CDR0000589150__1836/?report=objectonly" target="object" rid-figpopup="figCDR00005891501836" rid-ob="figobCDR00005891501836">Figure 2</a>). Additionally, their closeness to the optic nerves and chiasm may result in vision problems. Some patients present with obstructive hydrocephalus caused by tumor growth within the third ventricle. Rarely, tumors may extend into the posterior fossa, and patients may present with headache, diplopia, ataxia, and hearing loss.[<a class="bk_pop" href="#CDR0000589150_rl_4_5">5</a>]</p><div id="CDR0000589150__1836" class="figure bk_fig"><div class="graphic"><a href="/core/lw/2.0/html/tileshop_pmc/tileshop_pmc_inline.html?title=Figure%202&p=BOOKS&id=567186_CDR0000803492.jpg" target="tileshopwindow" class="inline_block pmc_inline_block ts_canvas img_link" title="Click on image to zoom"><div class="ts_bar small" title="Click on image to zoom"></div><img src="/books/NBK65891.14/bin/CDR0000803492.jpg" alt="Drawing showing a coronal view of the inside of the brain where craniopharyngiomas may form. A tumor is shown between the pituitary gland and the optic chiasm. Also shown is the oculomotor nerve (III), trochlear nerve (IV), abducens nerve (VI), ophthalmic nerve (V1), maxillary nerve (V2), internal carotid artery, and the cavernous and sphenoid sinuses." class="tileshop" title="Click on image to zoom" /></a></div><div class="caption"><p>Figure 2. Drawing showing a coronal view of the inside of the brain where craniopharyngiomas may form. The tumor usually occurs in the region of the pituitary gland, near the optic chiasm and optic nerves.</p></div></div></div><div id="CDR0000589150__141"><h3>Diagnostic Evaluation</h3><p id="CDR0000589150__142">CT scans and MRI scans are often diagnostic for childhood craniopharyngiomas, with most tumors demonstrating intratumoral calcifications and a solid and cystic component. MRI of the spinal axis is not routinely performed.</p><p id="CDR0000589150__143">Craniopharyngiomas without calcification may be confused with other tumor types, including germ cell tumors, hypothalamic/chiasmatic astrocytomas, or Langerhans cell histiocytosis. Biopsy or resection is required to confirm the diagnosis.[<a class="bk_pop" href="#CDR0000589150_rl_4_6">6</a>] </p><p id="CDR0000589150__145">Apart from imaging, patients often undergo endocrine testing and formal vision examination, including visual-field evaluation.</p></div><div id="CDR0000589150__55"><h3>Prognosis</h3><p id="CDR0000589150__56">Regardless of the treatment modality, the long-term event-free survival rate is approximately 65% in children,[<a class="bk_pop" href="#CDR0000589150_rl_4_3">3</a>,<a class="bk_pop" href="#CDR0000589150_rl_4_4">4</a>] with 5-year and 10-year overall survival rates higher than 90% in children between the ages of 0 and 14 years.[<a class="bk_pop" href="#CDR0000589150_rl_4_1">1</a>,<a class="bk_pop" href="#CDR0000589150_rl_4_7">7</a>-<a class="bk_pop" href="#CDR0000589150_rl_4_9">9</a>]</p></div><div id="CDR0000589150_rl_4"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000589150_rl_4_1">Ostrom QT, Cioffi G, Gittleman H, et al.: CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2012-2016. Neuro Oncol 21 (Suppl 5): v1-v100, 2019. [<a href="/pmc/articles/PMC6823730/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6823730</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/31675094" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 31675094</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_4_2">Louis DN, Ohgaki H, Wiestler OD: WHO Classification of Tumours of the Central Nervous System. 4th rev.ed. IARC Press, 2016.</div></li><li><div class="bk_ref" id="CDR0000589150_rl_4_3">Karavitaki N, Wass JA: Craniopharyngiomas. Endocrinol Metab Clin North Am 37 (1): 173-93, ix-x, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18226736" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18226736</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_4_4">Garnett MR, Puget S, Grill J, et al.: Craniopharyngioma. Orphanet J Rare Dis 2: 18, 2007. [<a href="/pmc/articles/PMC1855047/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC1855047</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/17425791" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17425791</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_4_5">Zhou L, Luo L, Xu J, et al.: Craniopharyngiomas in the posterior fossa: a rare subgroup, diagnosis, management and outcomes. J Neurol Neurosurg Psychiatry 80 (10): 1150-4, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19762904" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19762904</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_4_6">Rossi A, Cama A, Consales A, et al.: Neuroimaging of pediatric craniopharyngiomas: a pictorial essay. J Pediatr Endocrinol Metab 19 (Suppl 1): 299-319, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16700305" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16700305</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_4_7">Muller HL: Childhood craniopharyngioma. Recent advances in diagnosis, treatment and follow-up. Horm Res 69 (4): 193-202, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18204266" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18204266</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_4_8">Müller HL: Childhood craniopharyngioma--current concepts in diagnosis, therapy and follow-up. Nat Rev Endocrinol 6 (11): 609-18, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20877295" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20877295</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_4_9">Zacharia BE, Bruce SS, Goldstein H, et al.: Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program. Neuro Oncol 14 (8): 1070-8, 2012. [<a href="/pmc/articles/PMC3408265/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3408265</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/22735773" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22735773</span></a>]</div></li></ol></div></div><div id="CDR0000589150__42"><h2 id="_CDR0000589150__42_"> Histopathologic Classification of Childhood Craniopharyngioma</h2><p id="CDR0000589150__43">Craniopharyngiomas are histologically benign and often occur in the suprasellar region, with an intrasellar portion. They may be locally invasive and typically do not metastasize to remote brain locations. </p><p id="CDR0000589150__178">Craniopharyngiomas are classified as one of the following:</p><ul id="CDR0000589150__148"><li class="half_rhythm"><div><b>Adamantinomatous:</b> Adamantinomatous craniopharyngioma is the type found most frequently in children.[<a class="bk_pop" href="#CDR0000589150_rl_42_1">1</a>] These tumors are typically composed of a solid portion formed by nests and trabeculae of epithelial tumor cells, with an abundance of calcification, and a cystic component that is filled with a dark, oily fluid. Wet keratin is also characteristic. Adamantinomatous craniopharyngiomas are more locally aggressive than are papillary tumors and have a significantly higher rate of recurrence.[<a class="bk_pop" href="#CDR0000589150_rl_42_2">2</a>] Activating <i>CTNNB1</i> gene mutations are found in most adamantinomatous tumors.[<a class="bk_pop" href="#CDR0000589150_rl_42_3">3</a>-<a class="bk_pop" href="#CDR0000589150_rl_42_5">5</a>]</div></li><li class="half_rhythm"><div><b>Papillary:</b> Papillary craniopharyngiomas occur primarily in adults. <i>BRAF</i> V600E mutations are observed in nearly all papillary craniopharyngiomas.[<a class="bk_pop" href="#CDR0000589150_rl_42_4">4</a>,<a class="bk_pop" href="#CDR0000589150_rl_42_5">5</a>]</div></li></ul><div id="CDR0000589150_rl_42"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000589150_rl_42_1">Karavitaki N, Wass JA: Craniopharyngiomas. Endocrinol Metab Clin North Am 37 (1): 173-93, ix-x, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18226736" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18226736</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_42_2">Pekmezci M, Louie J, Gupta N, et al.: Clinicopathological characteristics of adamantinomatous and papillary craniopharyngiomas: University of California, San Francisco experience 1985-2005. Neurosurgery 67 (5): 1341-9; discussion 1349, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20871436" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20871436</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_42_3">Sekine S, Shibata T, Kokubu A, et al.: Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol 161 (6): 1997-2001, 2002. [<a href="/pmc/articles/PMC1850925/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC1850925</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/12466115" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 12466115</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_42_4">Brastianos PK, Taylor-Weiner A, Manley PE, et al.: Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas. Nat Genet 46 (2): 161-5, 2014. [<a href="/pmc/articles/PMC3982316/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3982316</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/24413733" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24413733</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_42_5">Goschzik T, Gessi M, Dreschmann V, et al.: Genomic Alterations of Adamantinomatous and Papillary Craniopharyngioma. J Neuropathol Exp Neurol 76 (2): 126-134, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28069929" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28069929</span></a>]</div></li></ol></div></div><div id="CDR0000589150__9"><h2 id="_CDR0000589150__9_">Stage Information for Childhood Craniopharyngioma</h2><p id="CDR0000589150__10">There is no generally applied staging system for childhood craniopharyngiomas. For treatment purposes, patients are grouped as having newly diagnosed or recurrent disease.</p></div><div id="CDR0000589150__168"><h2 id="_CDR0000589150__168_">Treatment Option Overview for Childhood Craniopharyngioma</h2><p id="CDR0000589150__198"><a class="figpopup" href="/books/NBK65891.14/table/CDR0000589150__169/?report=objectonly" target="object" rid-figpopup="figCDR0000589150169" rid-ob="figobCDR0000589150169">Table 1</a> describes the treatment options for newly diagnosed and recurrent childhood craniopharyngioma.</p><div id="CDR0000589150__169" class="table"><h3><span class="title">Table 1. Treatment Options for Childhood Craniopharyngioma</span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK65891.14/table/CDR0000589150__169/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000589150__169_lrgtbl__"><table class="no_top_margin"><thead><tr><th colspan="1" rowspan="1" style="vertical-align:top;">Treatment Group</th><th colspan="1" rowspan="1" style="vertical-align:top;">Treatment Options</th></tr></thead><tbody><tr><td colspan="1" rowspan="4" style="vertical-align:top;">Newly diagnosed childhood craniopharyngioma</td><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000589150__13">Complete resection with or without radiation therapy</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000589150__18">Subtotal resection with radiation therapy</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000589150__74">Primary cyst drainage with or without radiation therapy</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000589150__1821">Intracystic therapy (i.e., interferon-alpha)</a></td></tr><tr><td colspan="1" rowspan="4" style="vertical-align:top;">Recurrent childhood craniopharyngioma</td><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000589150__160">Surgery</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000589150__160">Radiation therapy, including radiosurgery</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000589150__160">Intracavitary instillation of radioactive phosphorus P 32, bleomycin, or interferon-alpha, for those with cystic recurrences</a></td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;"><a href="#CDR0000589150__160">Systemic peginterferon alpha-2b</a></td></tr></tbody></table></div></div><p id="CDR0000589150__1799">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="#CDR0000589150_rl_168_1">1</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><div id="CDR0000589150_rl_168"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000589150_rl_168_1">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></ol></div></div><div id="CDR0000589150__11"><h2 id="_CDR0000589150__11_">Newly Diagnosed Childhood Craniopharyngioma Treatment</h2><div id="CDR0000589150__166"><h3>Treatment Options for Newly Diagnosed Childhood Craniopharyngioma</h3><p id="CDR0000589150__60">There is no consensus on the optimal treatment for patients with newly diagnosed craniopharyngioma, in part because of the lack of prospective randomized trials that compare the different treatment options. Treatment is individualized on the basis of factors that include the following:</p><ul id="CDR0000589150__149"><li class="half_rhythm"><div>Tumor size.</div></li><li class="half_rhythm"><div>Tumor location.</div></li><li class="half_rhythm"><div>Extension of the tumor.</div></li><li class="half_rhythm"><div>Potential short-term and long-term toxicity.</div></li></ul><p id="CDR0000589150__150">Treatment options for newly diagnosed childhood craniopharyngioma include the following:</p><ol id="CDR0000589150__170"><li class="half_rhythm"><div><a href="#CDR0000589150__13">Complete resection with or without radiation therapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000589150__18">Subtotal resection with radiation therapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000589150__74">Primary cyst drainage with or without radiation therapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000589150__1821">Intracystic therapy (i.e., interferon-alpha)</a>.</div></li></ol><div id="CDR0000589150__13"><h4>Complete resection with or without radiation therapy</h4><p id="CDR0000589150__14">It may be possible to remove all visible tumor and achieve long-term disease control.[<a class="bk_pop" href="#CDR0000589150_rl_11_1">1</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335138/" class="def">Level of evidence: 3iA</a>]; [<a class="bk_pop" href="#CDR0000589150_rl_11_2">2</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335151/" class="def">Level of evidence: 3iiiB</a>]; [<a class="bk_pop" href="#CDR0000589150_rl_11_3">3</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335152/" class="def">Level of evidence: 3iiiC</a>] A 5-year progression-free survival (PFS) rate of about 65% has been reported.[<a class="bk_pop" href="#CDR0000589150_rl_11_4">4</a>] Gross-total resection is often technically challenging because the tumor is surrounded by vital structures, including the optic nerves and chiasm, the carotid artery and its branches, the hypothalamus, and the third cranial nerve. These structures may limit the ability to remove the entire tumor.</p><p id="CDR0000589150__153">Many surgical approaches have been described, and the choice is determined by tumor size, location, and extension. Surgical approaches include the following:</p><ul id="CDR0000589150__154"><li class="half_rhythm"><div class="half_rhythm"><b>Craniotomy:</b> As noted above, gross-total resection may be technically challenging because the tumor is surrounded by vital structures. The surgeon often has a limited view of the hypothalamic and sellar regions, and portions of the mass may remain after surgery, accounting for some recurrences. An understanding of the complex variations in how the tumors grow anatomically may help facilitate gross-total resection.[<a class="bk_pop" href="#CDR0000589150_rl_11_5">5</a>] Nonetheless, almost all craniopharyngiomas have an attachment to the pituitary stalk, and of the patients who undergo complete resection, virtually all will require lifelong pituitary hormone replacement with multiple medications.[<a class="bk_pop" href="#CDR0000589150_rl_11_2">2</a>,<a class="bk_pop" href="#CDR0000589150_rl_11_6">6</a>]</div><div class="half_rhythm">Complications of complete resection include the following:<dl id="CDR0000589150__180" class="temp-labeled-list"><dt>-</dt><dd><p class="no_top_margin">Obesity, which can be life-threatening.[<a class="bk_pop" href="#CDR0000589150_rl_11_7">7</a>]</p></dd><dt>-</dt><dd><p class="no_top_margin">Need for hormone replacement therapy.[<a class="bk_pop" href="#CDR0000589150_rl_11_8">8</a>]</p></dd><dt>-</dt><dd><p class="no_top_margin">Severe behavioral problems.[<a class="bk_pop" href="#CDR0000589150_rl_11_8">8</a>]</p></dd><dt>-</dt><dd><p class="no_top_margin">Blindness.</p></dd><dt>-</dt><dd><p class="no_top_margin">Seizures.</p></dd><dt>-</dt><dd><p class="no_top_margin">Spinal fluid leak.</p></dd><dt>-</dt><dd><p class="no_top_margin">False aneurysms.</p></dd><dt>-</dt><dd><p class="no_top_margin">Difficulty with eye movements. </p></dd><dt>-</dt><dd><p class="no_top_margin">Death from intraoperative hemorrhage, hypothalamic damage, or stroke (rare). </p></dd></dl></div></li><li class="half_rhythm"><div class="half_rhythm"><b>Transsphenoidal approach:</b> A transsphenoidal approach may be possible for some small tumors located entirely within the sella.[<a class="bk_pop" href="#CDR0000589150_rl_11_9">9</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335152/" class="def">Level of evidence: 3iiiC</a>] The development of expanded endonasal techniques with endoscopic visualization have allowed increased use of this approach, even for sizeable childhood tumors, which is similar to the experience in adults.[<a class="bk_pop" href="#CDR0000589150_rl_11_10">10</a>] A complete resection can be obtained using this approach, with associated complications of panhypopituitarism and the risk of cerebrospinal fluid leaks.[<a class="bk_pop" href="#CDR0000589150_rl_11_11">11</a>] When an endonasal approach is not possible, a craniotomy is required.</div></li></ul><p id="CDR0000589150__17">If the surgeon indicates that the tumor was not completely removed or if postoperative imaging reveals residual craniopharyngioma, radiation therapy may be recommended to prevent early progression (refer to the <a href="#CDR0000589150__18">Subtotal resection with radiation therapy</a> section of this summary for more information).[<a class="bk_pop" href="#CDR0000589150_rl_11_12">12</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>] </p><p id="CDR0000589150__156">Periodic surveillance using magnetic resonance imaging is performed for several years after complete resection because of the possibility of tumor recurrence.</p></div><div id="CDR0000589150__18"><h4>Subtotal resection with radiation therapy</h4><p id="CDR0000589150__19">The goal of limited surgery is to establish a diagnosis, drain any cysts, and decompress the optic nerves. No attempt is made to remove tumor from the pituitary stalk or hypothalamus in an effort to minimize the late effects associated with complete resection.[<a class="bk_pop" href="#CDR0000589150_rl_11_13">13</a>] </p><p id="CDR0000589150__181">The surgical procedure is often followed by radiation therapy, with a 5-year PFS rate of about 70% to 90% [<a class="bk_pop" href="#CDR0000589150_rl_11_4">4</a>,<a class="bk_pop" href="#CDR0000589150_rl_11_14">14</a>]; [<a class="bk_pop" href="#CDR0000589150_rl_11_15">15</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335143/" class="def">Level of evidence: 3iDiii</a>] and 10-year overall survival rates exceeding 90%.[<a class="bk_pop" href="#CDR0000589150_rl_11_16">16</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335144/" class="def">Level of evidence: 3iiA</a>]; [<a class="bk_pop" href="#CDR0000589150_rl_11_17">17</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>] The standard approach to radiation therapy involves fractionated external-beam radiation, with a recommended dose of 50 Gy to 54 Gy, in 1.8-Gy fractions, restricting the optic chiasm dose to 54 Gy.[<a class="bk_pop" href="#CDR0000589150_rl_11_18">18</a>-<a class="bk_pop" href="#CDR0000589150_rl_11_21">21</a>] Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention.[<a class="bk_pop" href="#CDR0000589150_rl_11_22">22</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000587989/" class="def">Level of evidence: 3iDiv</a>]</p><p id="CDR0000589150__152">A systematic review of 109 reports that described extent of resection found that subtotal resection plus radiation therapy was associated with rates of tumor control similar to those for gross-total resection. It was also reported that both approaches were associated with PFS rates higher than those for subtotal resection alone.[<a class="bk_pop" href="#CDR0000589150_rl_11_17">17</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>] </p><p id="CDR0000589150__158"> Surgical complications with a subtotal resection are less likely than with complete resection. Complications of radiation therapy include the following:</p><ul id="CDR0000589150__182"><li class="half_rhythm"><div>Loss of pituitary hormonal function.</div></li><li class="half_rhythm"><div>Cognitive dysfunction.</div></li><li class="half_rhythm"><div>Development of late strokes and vascular malformations.</div></li><li class="half_rhythm"><div>Delayed blindness.</div></li><li class="half_rhythm"><div>Development of second tumors.</div></li><li class="half_rhythm"><div>Malignant transformation of the primary tumor within the radiation field (rare).[<a class="bk_pop" href="#CDR0000589150_rl_11_23">23</a>,<a class="bk_pop" href="#CDR0000589150_rl_11_24">24</a>]</div></li></ul><p id="CDR0000589150__183">Newer radiation technologies such as intensity-modulated photon therapy and proton therapy may reduce the radiation dose to uninvolved parts of the brain and spare normal tissue. When these highly conformal radiation treatments are employed, interim imaging is commonly performed to detect changes in cyst volume, with treatment plans modified as appropriate.[<a class="bk_pop" href="#CDR0000589150_rl_11_21">21</a>,<a class="bk_pop" href="#CDR0000589150_rl_11_25">25</a>,<a class="bk_pop" href="#CDR0000589150_rl_11_26">26</a>] It is unknown whether such technologies reduce late effects from radiation.[<a class="bk_pop" href="#CDR0000589150_rl_11_15">15</a>,<a class="bk_pop" href="#CDR0000589150_rl_11_21">21</a>,<a class="bk_pop" href="#CDR0000589150_rl_11_26">26</a>,<a class="bk_pop" href="#CDR0000589150_rl_11_27">27</a>]</p><p id="CDR0000589150__159">Tumor progression remains a concern, and it is usually not possible to repeat a full course of standard fractionated radiation. In selected cases, <a href="/books/n/pdqcis/glossary/def-item/glossary_CDR0000044464/" class="def">stereotactic radiation therapy</a> can be delivered as a single large dose of radiation to a small field.[<a class="bk_pop" href="#CDR0000589150_rl_11_28">28</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335140/" class="def">Level of evidence: 3iC</a>] Proximity of the craniopharyngioma to vital structures, particularly the optic nerves, limits this to small tumors within the sella.[<a class="bk_pop" href="#CDR0000589150_rl_11_29">29</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>]</p></div><div id="CDR0000589150__74"><h4>Primary cyst drainage with or without radiation therapy</h4><p id="CDR0000589150__75">For predominantly cystic craniopharyngiomas, stereotactic drainage of the cyst followed by radiation therapy may be a viable alternative treatment to attempted surgical resection. This procedure may also allow the surgeon to use a two-stage approach: first draining the cyst via the implanted catheter, to relieve pressure and complicating symptoms; and then later resecting the tumor or employing radiation therapy.[<a class="bk_pop" href="#CDR0000589150_rl_11_30">30</a>]</p></div><div id="CDR0000589150__1821"><h4>Intracystic therapy</h4><p id="CDR0000589150__1822"> Intracystic interferon-alpha may also be a treatment option for primary cystic lesions, after ensuring the integrity of the cyst wall, to delay the need for alternative therapies.[<a class="bk_pop" href="#CDR0000589150_rl_11_31">31</a>]</p></div></div><div id="CDR0000589150__TrialSearch_11_sid_5"><h3>Current Clinical Trials</h3><p id="CDR0000589150__TrialSearch_11_22">Use our <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/advanced-search" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">advanced clinical trial search</a> to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">General information</a> about clinical trials is also available.</p></div><div id="CDR0000589150_rl_11"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000589150_rl_11_1">Mortini P, Losa M, Pozzobon G, et al.: Neurosurgical treatment of craniopharyngioma in adults and children: early and long-term results in a large case series. J Neurosurg 114 (5): 1350-9, 2011. 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[<a href="https://pubmed.ncbi.nlm.nih.gov/20121363" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20121363</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_11_25">Winkfield KM, Linsenmeier C, Yock TI, et al.: Surveillance of craniopharyngioma cyst growth in children treated with proton radiotherapy. Int J Radiat Oncol Biol Phys 73 (3): 716-21, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/18676089" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18676089</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_11_26">Beltran C, Roca M, Merchant TE: On the benefits and risks of proton therapy in pediatric craniopharyngioma. Int J Radiat Oncol Biol Phys 82 (2): e281-7, 2012. [<a href="/pmc/articles/PMC3554244/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3554244</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/21570209" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21570209</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_11_27">Boehling NS, Grosshans DR, Bluett JB, et al.: Dosimetric comparison of three-dimensional conformal proton radiotherapy, intensity-modulated proton therapy, and intensity-modulated radiotherapy for treatment of pediatric craniopharyngiomas. Int J Radiat Oncol Biol Phys 82 (2): 643-52, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/21277111" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21277111</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_11_28">Kobayashi T: Long-term results of gamma knife radiosurgery for 100 consecutive cases of craniopharyngioma and a treatment strategy. Prog Neurol Surg 22: 63-76, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/18948720" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18948720</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_11_29">Hasegawa T, Kobayashi T, Kida Y: Tolerance of the optic apparatus in single-fraction irradiation using stereotactic radiosurgery: evaluation in 100 patients with craniopharyngioma. Neurosurgery 66 (4): 688-94; discussion 694-5, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20190668" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20190668</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_11_30">Schubert T, Trippel M, Tacke U, et al.: Neurosurgical treatment strategies in childhood craniopharyngiomas: is less more? Childs Nerv Syst 25 (11): 1419-27, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19714341" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19714341</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_11_31">Kilday JP, Caldarelli M, Massimi L, et al.: Intracystic interferon-alpha in pediatric craniopharyngioma patients: an international multicenter assessment on behalf of SIOPE and ISPN. Neuro Oncol 19 (10): 1398-1407, 2017. [<a href="/pmc/articles/PMC5596165/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5596165</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/28499018" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28499018</span></a>]</div></li></ol></div></div><div id="CDR0000589150__26"><h2 id="_CDR0000589150__26_">Recurrent Childhood Craniopharyngioma Treatment</h2><div id="CDR0000589150__167"><h3>Treatment Options for Recurrent Childhood Craniopharyngioma</h3><p id="CDR0000589150__27">Recurrence of craniopharyngioma occurs in approximately 35% of patients regardless of primary therapy.[<a class="bk_pop" href="#CDR0000589150_rl_26_1">1</a>]</p><p id="CDR0000589150__160">Treatment options for recurrent childhood craniopharyngioma include the following:</p><ol id="CDR0000589150__171"><li class="half_rhythm"><div>Surgery.</div></li><li class="half_rhythm"><div>Radiation therapy, including <a href="/books/n/pdqcis/glossary/def-item/glossary_CDR0000046701/" class="def">radiosurgery</a>.</div></li><li class="half_rhythm"><div>Intracavitary instillation of radioactive phosphorus P 32 (32P), bleomycin, or interferon-alpha, for those with cystic recurrences.</div></li><li class="half_rhythm"><div>Systemic peginterferon alpha-2b.</div></li></ol><p id="CDR0000589150__172">The management of recurrent craniopharyngioma is determined largely by previous therapy. Repeat attempts at gross-total resections are difficult, and long-term disease control is achieved less often.[<a class="bk_pop" href="#CDR0000589150_rl_26_2">2</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335153/" class="def">Level of evidence: 3iiiDi</a>] Complications are more frequent than with initial surgery.[<a class="bk_pop" href="#CDR0000589150_rl_26_3">3</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335153/" class="def">Level of evidence: 3iiiDi</a>] If not previously employed, external-beam radiation therapy is an option, including consideration of <a href="/books/n/pdqcis/glossary/def-item/glossary_CDR0000046701/" class="def">radiosurgery</a> in selected circumstances.[<a class="bk_pop" href="#CDR0000589150_rl_26_4">4</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>]</p><p id="CDR0000589150__162">Cystic recurrences may be treated with intracavitary instillation of varying agents via placement of an Ommaya catheter.[<a class="bk_pop" href="#CDR0000589150_rl_26_5">5</a>] These agents have included radioactive 32P or other radioactive compounds,[<a class="bk_pop" href="#CDR0000589150_rl_26_6">6</a>-<a class="bk_pop" href="#CDR0000589150_rl_26_8">8</a>]; [<a class="bk_pop" href="#CDR0000589150_rl_26_9">9</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335132/" class="def">Level of evidence: 2A</a>] bleomycin,[<a class="bk_pop" href="#CDR0000589150_rl_26_10">10</a>]; [<a class="bk_pop" href="#CDR0000589150_rl_26_11">11</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>] or interferon-alpha.[<a class="bk_pop" href="#CDR0000589150_rl_26_12">12</a>]; [<a class="bk_pop" href="#CDR0000589150_rl_26_13">13</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335151/" class="def">Level of evidence: 3iiiB</a>]; [<a class="bk_pop" href="#CDR0000589150_rl_26_14">14</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335153/" class="def">Level of evidence: 3iiiDi</a>] These strategies have been found to be useful in certain cases, and a low risk of complications has been reported. However, none of these approaches have shown efficacy against solid portions of the tumor.</p><p id="CDR0000589150__163">Although systemic therapy is generally not utilized, a small series has shown that the use of subcutaneous peginterferon alpha-2b to manage cystic recurrences can result in durable responses.[<a class="bk_pop" href="#CDR0000589150_rl_26_15">15</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335158/" class="def">Level of evidence: 3iiiDiii</a>] In addition, case reports demonstrating dramatic tumor response to BRAF inhibitors in adults with papillary craniopharyngiomas suggest that there may be a role for BRAF inhibitor therapy in the rare setting of a child with a papillary craniopharyngioma.[<a class="bk_pop" href="#CDR0000589150_rl_26_16">16</a>,<a class="bk_pop" href="#CDR0000589150_rl_26_17">17</a>]</p></div><div id="CDR0000589150__111"><h3>Treatment Options Under Clinical Evaluation for Recurrent Childhood Craniopharyngioma</h3><p id="CDR0000589150__112">Early-phase therapeutic trials may be available for selected patients. These trials may be available via the Children’s Oncology Group (COG), the <a href="http://www.pbtc.org/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Pediatric Brain Tumor Consortium</a>, or other entities. 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="CDR0000589150__1800">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000589150__113"><li class="half_rhythm"><div class="half_rhythm"><b><a href="https://www.cancer.gov/clinicaltrials/NCT03155620" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">APEC1621 (NCT03155620)</a></b> (Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients with Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders)<b>:</b> NCI-COG Pediatric Molecular Analysis for Therapeutic Choice (MATCH), referred to as Pediatric MATCH, will match targeted agents with specific molecular changes identified using a next-generation sequencing targeted assay of more than 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&targetsite=external&targetcat=link&targettype=uri">NCI website</a> and <a href="https://clinicaltrials.gov/ct2/show/NCT03155620" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a>.
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</div></li></ul></div><div id="CDR0000589150_rl_26"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000589150_rl_26_1">Yang I, Sughrue ME, Rutkowski MJ, et al.: Craniopharyngioma: a comparison of tumor control with various treatment strategies. Neurosurg Focus 28 (4): E5, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20367362" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20367362</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_2">Vinchon M, Dhellemmes P: Craniopharyngiomas in children: recurrence, reoperation and outcome. Childs Nerv Syst 24 (2): 211-7, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/17786455" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17786455</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_3">Jang WY, Lee KS, Son BC, et al.: Repeat operations in pediatric patients with recurrent craniopharyngiomas. Pediatr Neurosurg 45 (6): 451-5, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/20110758" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20110758</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_4">Xu Z, Yen CP, Schlesinger D, et al.: Outcomes of Gamma Knife surgery for craniopharyngiomas. J Neurooncol 104 (1): 305-13, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21153860" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21153860</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_5">Steinbok P, Hukin J: Intracystic treatments for craniopharyngioma. Neurosurg Focus 28 (4): E13, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20367357" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20367357</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_6">Julow J, Backlund EO, Lányi F, et al.: Long-term results and late complications after intracavitary yttrium-90 colloid irradiation of recurrent cystic craniopharyngiomas. Neurosurgery 61 (2): 288-95; discussion 295-6, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17762741" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17762741</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_7">Barriger RB, Chang A, Lo SS, et al.: Phosphorus-32 therapy for cystic craniopharyngiomas. Radiother Oncol 98 (2): 207-12, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21269713" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21269713</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_8">Maarouf M, El Majdoub F, Fuetsch M, et al.: Stereotactic intracavitary brachytherapy with P-32 for cystic craniopharyngiomas in children. Strahlenther Onkol 192 (3): 157-65, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26541336" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26541336</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_9">Kickingereder P, Maarouf M, El Majdoub F, et al.: Intracavitary brachytherapy using stereotactically applied phosphorus-32 colloid for treatment of cystic craniopharyngiomas in 53 patients. J Neurooncol 109 (2): 365-74, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22717668" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22717668</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_10">Linnert M, Gehl J: Bleomycin treatment of brain tumors: an evaluation. Anticancer Drugs 20 (3): 157-64, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19396014" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19396014</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_11">Hukin J, Steinbok P, Lafay-Cousin L, et al.: Intracystic bleomycin therapy for craniopharyngioma in children: the Canadian experience. Cancer 109 (10): 2124-31, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17407137" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17407137</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_12">Ierardi DF, Fernandes MJ, Silva IR, et al.: Apoptosis in alpha interferon (IFN-alpha) intratumoral chemotherapy for cystic craniopharyngiomas. Childs Nerv Syst 23 (9): 1041-6, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17593372" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17593372</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_13">Cavalheiro S, Di Rocco C, Valenzuela S, et al.: Craniopharyngiomas: intratumoral chemotherapy with interferon-alpha: a multicenter preliminary study with 60 cases. Neurosurg Focus 28 (4): E12, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20367356" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20367356</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_14">Kilday JP, Caldarelli M, Massimi L, et al.: Intracystic interferon-alpha in pediatric craniopharyngioma patients: an international multicenter assessment on behalf of SIOPE and ISPN. Neuro Oncol 19 (10): 1398-1407, 2017. [<a href="/pmc/articles/PMC5596165/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5596165</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/28499018" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28499018</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_15">Yeung JT, Pollack IF, Panigrahy A, et al.: Pegylated interferon-α-2b for children with recurrent craniopharyngioma. J Neurosurg Pediatr 10 (6): 498-503, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/23061825" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23061825</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_16">Aylwin SJ, Bodi I, Beaney R: Pronounced response of papillary craniopharyngioma to treatment with vemurafenib, a BRAF inhibitor. Pituitary 19 (5): 544-6, 2016. [<a href="/pmc/articles/PMC4996872/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4996872</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/26115708" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26115708</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_26_17">Roque A, Odia Y: BRAF-V600E mutant papillary craniopharyngioma dramatically responds to combination BRAF and MEK inhibitors. CNS Oncol 6 (2): 95-99, 2017. [<a href="/pmc/articles/PMC6020871/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6020871</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/28425764" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28425764</span></a>]</div></li></ol></div></div><div id="CDR0000589150__28"><h2 id="_CDR0000589150__28_">Late Effects in Patients Treated for Childhood Craniopharyngioma </h2><p id="CDR0000589150__30">Quality-of-life issues are important in this group of patients and are difficult to generalize because of the various treatment modalities. In one series of 261 patients diagnosed with craniopharyngiomas before 2000, hypothalamic involvement was associated with lower overall survival (OS), impaired quality-of-life, and severe obesity.[<a class="bk_pop" href="#CDR0000589150_rl_28_1">1</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335138/" class="def">Level of evidence: 3iA</a>]</p><p id="CDR0000589150__174">Late effects of treatment for childhood craniopharyngioma include the following:</p><ul id="CDR0000589150__173"><li class="half_rhythm"><div>Behavioral issues and memory deficits. Although intelligence quotient is usually maintained, behavioral issues and memory deficits attributed to the frontal lobe and hypothalamus commonly occur.[<a class="bk_pop" href="#CDR0000589150_rl_28_2">2</a>,<a class="bk_pop" href="#CDR0000589150_rl_28_3">3</a>] Patients with hypothalamic involvement showed impairment in memory and executive functioning.[<a class="bk_pop" href="#CDR0000589150_rl_28_4">4</a>]</div></li><li class="half_rhythm"><div>Visual disturbances, including visual field and acuity defects.[<a class="bk_pop" href="#CDR0000589150_rl_28_5">5</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335152/" class="def">Level of evidence: 3iiiC</a>]</div></li><li class="half_rhythm"><div> Endocrine abnormalities. Endocrine abnormalities result in the almost universal need for lifelong endocrine replacement with multiple pituitary hormones.[<a class="bk_pop" href="#CDR0000589150_rl_28_3">3</a>]; [<a class="bk_pop" href="#CDR0000589150_rl_28_6">6</a>-<a class="bk_pop" href="#CDR0000589150_rl_28_8">8</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335152/" class="def">Level of evidence: 3iiiC</a>] A report indicated that adults, despite being treated with long-term growth hormone replacement after childhood-onset craniopharyngioma involving the hypothalamus, were at increased risk of developing cardiovascular disease.[<a class="bk_pop" href="#CDR0000589150_rl_28_9">9</a>] </div></li><li class="half_rhythm"><div>Decreased height. Growth hormone replacement initiated in childhood results in increases in height without impact on OS and progression-free survival when compared with children who did not receive growth hormone.[<a class="bk_pop" href="#CDR0000589150_rl_28_10">10</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335152/" class="def">Level of evidence: 3iiiC</a>] Growth hormone administration beginning 1 year after diagnosis may be associated with early improvements in quality of life when measured at 3 years postdiagnosis.[<a class="bk_pop" href="#CDR0000589150_rl_28_11">11</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335140/" class="def">Level of evidence: 3iC</a>]</div></li><li class="half_rhythm"><div>Obesity, which can be life-threatening, and the development of metabolic syndrome, including nonalcoholic fatty liver disease.[<a class="bk_pop" href="#CDR0000589150_rl_28_12">12</a>,<a class="bk_pop" href="#CDR0000589150_rl_28_13">13</a>] Children who undergo complete resection or subtotal resection may develop obesity, suggesting that a predilection to obesity may be a component of the disease itself, as opposed to the result of direct hypothalamic injury.[<a class="bk_pop" href="#CDR0000589150_rl_28_14">14</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335140/" class="def">Level of evidence: 3iC</a>]</div></li><li class="half_rhythm"><div>Vasculopathies and stroke. Vasculopathies and subsequent strokes may result from local irradiation.[<a class="bk_pop" href="#CDR0000589150_rl_28_15">15</a>,<a class="bk_pop" href="#CDR0000589150_rl_28_16">16</a>] One cross-sectional cohort study observed a trend suggesting that long-term growth hormone replacement may reduce the risk of stroke.[<a class="bk_pop" href="#CDR0000589150_rl_28_16">16</a>]</div></li><li class="half_rhythm"><div>Subsequent neoplasms. Subsequent neoplasms may result from local irradiation.[<a class="bk_pop" href="#CDR0000589150_rl_28_15">15</a>]</div></li></ul><p id="CDR0000589150__54">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><div id="CDR0000589150_rl_28"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000589150_rl_28_1">Sterkenburg AS, Hoffmann A, Gebhardt U, et al.: Survival, hypothalamic obesity, and neuropsychological/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes. Neuro Oncol 17 (7): 1029-38, 2015. [<a href="/pmc/articles/PMC5654354/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5654354</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/25838139" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25838139</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_2">Winkfield KM, Tsai HK, Yao X, et al.: Long-term clinical outcomes following treatment of childhood craniopharyngioma. Pediatr Blood Cancer 56 (7): 1120-6, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21488157" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21488157</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_3">Giese H, Haenig B, Haenig A, et al.: Neurological and neuropsychological outcome after resection of craniopharyngiomas. J Neurosurg 132 (5): 1425-1434, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/31003210" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 31003210</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_4">Özyurt J, Thiel CM, Lorenzen A, et al.: Neuropsychological outcome in patients with childhood craniopharyngioma and hypothalamic involvement. J Pediatr 164 (4): 876-881.e4, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/24507865" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24507865</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_5">Wan MJ, Zapotocky M, Bouffet E, et al.: Long-term visual outcomes of craniopharyngioma in children. J Neurooncol 137 (3): 645-651, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/29344823" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29344823</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_6">Vinchon M, Weill J, Delestret I, et al.: Craniopharyngioma and hypothalamic obesity in children. Childs Nerv Syst 25 (3): 347-52, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19057910" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19057910</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_7">Dolson EP, Conklin HM, Li C, et al.: Predicting behavioral problems in craniopharyngioma survivors after conformal radiation therapy. Pediatr Blood Cancer 52 (7): 860-4, 2009. [<a href="/pmc/articles/PMC2727936/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC2727936</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/19191345" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19191345</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_8">Kawamata T, Amano K, Aihara Y, et al.: Optimal treatment strategy for craniopharyngiomas based on long-term functional outcomes of recent and past treatment modalities. Neurosurg Rev 33 (1): 71-81, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/19669813" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19669813</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_9">Holmer H, Ekman B, Björk J, et al.: Hypothalamic involvement predicts cardiovascular risk in adults with childhood onset craniopharyngioma on long-term GH therapy. Eur J Endocrinol 161 (5): 671-9, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/19667040" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19667040</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_10">Boekhoff S, Bogusz A, Sterkenburg AS, et al.: Long-term Effects of Growth Hormone Replacement Therapy in Childhood-onset Craniopharyngioma: Results of the German Craniopharyngioma Registry (HIT-Endo). Eur J Endocrinol 179 (5): 331-341, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/30139824" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30139824</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_11">Heinks K, Boekhoff S, Hoffmann A, et al.: Quality of life and growth after childhood craniopharyngioma: results of the multinational trial KRANIOPHARYNGEOM 2007. Endocrine 59 (2): 364-372, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/29230635" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29230635</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_12">Elowe-Gruau E, Beltrand J, Brauner R, et al.: Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity. J Clin Endocrinol Metab 98 (6): 2376-82, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/23633208" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 23633208</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_13">Hoffmann A, Bootsveld K, Gebhardt U, et al.: Nonalcoholic fatty liver disease and fatigue in long-term survivors of childhood-onset craniopharyngioma. Eur J Endocrinol 173 (3): 389-97, 2015. [<a href="https://pubmed.ncbi.nlm.nih.gov/26088821" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26088821</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_14">Tan TS, Patel L, Gopal-Kothandapani JS, et al.: The neuroendocrine sequelae of paediatric craniopharyngioma: a 40-year meta-data analysis of 185 cases from three UK centres. Eur J Endocrinol 176 (3): 359-369, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28073908" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28073908</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_15">Kiehna EN, Merchant TE: Radiation therapy for pediatric craniopharyngioma. Neurosurg Focus 28 (4): E10, 2010. [<a href="https://pubmed.ncbi.nlm.nih.gov/20367354" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20367354</span></a>]</div></li><li><div class="bk_ref" id="CDR0000589150_rl_28_16">Lo AC, Howard AF, Nichol A, et al.: A Cross-Sectional Cohort Study of Cerebrovascular Disease and Late Effects After Radiation Therapy for Craniopharyngioma. Pediatr Blood Cancer 63 (5): 786-93, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26756999" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26756999</span></a>]</div></li></ol></div></div><div id="CDR0000589150__47"><h2 id="_CDR0000589150__47_">Changes to This Summary (01/26/2021)</h2><p id="CDR0000589150__62">The PDQ cancer information summaries are reviewed regularly and updated as
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new information becomes available. This section describes the latest
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changes made to this summary as of the date above.</p><p id="CDR0000589150__1837">Added <a class="figpopup" href="/books/NBK65891.14/figure/CDR0000589150__1836/?report=objectonly" target="object" rid-figpopup="figCDR00005891501836" rid-ob="figobCDR00005891501836">Figure 2</a> depicting a coronal view of the inside of the brain where craniopharyngiomas may form. The tumor usually occurs in the region of the pituitary gland, near the optic chiasm and optic nerves.</p><p id="CDR0000589150__disclaimerHP_3">This summary is written and maintained by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric Treatment Editorial Board</a>, which is
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editorially independent of NCI. The summary reflects an independent review of
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the literature and does not represent a policy statement of NCI or NIH. More
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information about summary policies and the role of the PDQ Editorial Boards in
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maintaining the PDQ summaries can be found on the <a href="#CDR0000589150__AboutThis_1">About This PDQ Summary</a> and <a href="https://www.cancer.gov/publications/pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ® - NCI's Comprehensive Cancer Database</a> pages.
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</p></div><div id="CDR0000589150__AboutThis_1"><h2 id="_CDR0000589150__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000589150__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000589150__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood craniopharyngioma. 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="CDR0000589150__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000589150__AboutThis_5">This summary is reviewed regularly and updated as necessary by the <a href="https://www.cancer.gov/publications/pdq/editorial-boards/pediatric-treatment" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">PDQ Pediatric Treatment Editorial Board</a>, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).</p><p id="CDR0000589150__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000589150__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="CDR0000589150__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 Craniopharyngioma Treatment are:</p><ul><li class="half_rhythm"><div>Kenneth J. Cohen, MD, MBA (Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Hospital)</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>Roger J. Packer, MD (Children's National Hospital)</div></li><li class="half_rhythm"><div>D. Williams Parsons, MD, PhD</div></li><li class="half_rhythm"><div>Malcolm A. Smith, MD, PhD (National Cancer Institute)</div></li></ul><p id="CDR0000589150__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="CDR0000589150__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000589150__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="CDR0000589150__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000589150__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="CDR0000589150__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000589150__AboutThis_15">PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Craniopharyngioma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq</a>. Accessed <MM/DD/YYYY>. [PMID: 26389330]</p><p id="CDR0000589150__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="CDR0000589150__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000589150__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="CDR0000589150__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000589150__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 style="display:none"><div style="display:none" id="figCDR00005891501836"><img alt="Image CDR0000803492" src-large="/books/NBK65891.14/bin/CDR0000803492.jpg" /></div></div></div></div>
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Childhood Craniopharyngioma Treatment (PDQ®): Health Professional Version. 2021 Jan 26. In: PDQ Cancer Information Summaries [Internet]. 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ref="log$=inpage&link_id=inpage">Recurrent Childhood Craniopharyngioma Treatment</a></li><li><a href="#CDR0000589150__28" ref="log$=inpage&link_id=inpage">Late Effects in Patients Treated for Childhood Craniopharyngioma </a></li><li><a href="#CDR0000589150__47" ref="log$=inpage&link_id=inpage">Changes to This Summary (01/26/2021)</a></li><li><a href="#CDR0000589150__AboutThis_1" ref="log$=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/NBK65796/">Patient Version</a></li></ul></div></div><div class="portlet"><div 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</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/26389199" ref="ordinalpos=1&linkpos=2&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Hypopharyngeal Cancer Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Hypopharyngeal Cancer Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Adult Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/39591488" ref="ordinalpos=1&linkpos=3&log$=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> PALB2: Cancer Risks and Management (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> PALB2: Cancer Risks and Management (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em 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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 Adult 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/38113349" 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> RUNX1-Familial Platelet Disorder (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" 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