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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="#__NBK547409_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK547409_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="_NBK547409_"><span class="title" itemprop="name">Childhood Mesothelioma 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: May 19, 2023.</p><p class="small">Created: <span itemprop="datePublished">October 2, 2019</span>.</p></div><div class="jig-ncbiinpagenav body-content whole_rhythm" data-jigconfig="allHeadingLevels: ['h2'],smoothScroll: false" itemprop="text"><div id="_abs_rndgid_" itemprop="description"><p id="CDR0000799420__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood mesothelioma. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.</p><p id="CDR0000799420__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="CDR0000799420__1075"><h2 id="_CDR0000799420__1075_">Incidence, Risk Factors, and Clinical Presentation</h2><p id="CDR0000799420__1076">Mesothelioma is extremely rare in children and adolescents, with only 2% to 5% of patients presenting during the first two decades of life.[<a class="bk_pop" href="#CDR0000799420_rl_1075_1">1</a>] Fewer than 300 cases in children have been reported.[<a class="bk_pop" href="#CDR0000799420_rl_1075_2">2</a>]</p><p id="CDR0000799420__2461"> In adults, increased mesothelioma risk is associated with inherited <i>BAP1</i> mutations, exposures to asbestos, and exposures to radiation therapy during previous cancer treatments. These risk factors are rare in pediatric patients, and there are limited data that address cancer risk in children with asbestos exposures. The amount of radiation exposure required to develop cancer is
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also unknown.[<a class="bk_pop" href="#CDR0000799420_rl_1075_3">3</a>-<a class="bk_pop" href="#CDR0000799420_rl_1075_8">8</a>] </p><p id="CDR0000799420__2462">Mesothelioma may present in the thoracic/pleural region or in the peritoneum. These presentations have different clinical courses and prognoses. This cancer can involve the membranous coverings of the lung, the heart, or the
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abdominal organs.[<a class="bk_pop" href="#CDR0000799420_rl_1075_9">9</a>-<a class="bk_pop" href="#CDR0000799420_rl_1075_11">11</a>]; [<a class="bk_pop" href="#CDR0000799420_rl_1075_12">12</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810035/" class="def">Level of evidence C1</a>] Mesothelioma can spread onto organ surfaces
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without invading far into the underlying tissue. This cancer may also spread to regional or
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distant lymph nodes. </p><p id="CDR0000799420__1079">Benign and malignant mesotheliomas cannot be differentiated using histological
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criteria. Benign mesotheliomas are exceedingly rare and often occur in the peritoneal cavity. A poor prognosis is associated with mesotheliomas that are diffuse and
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invasive or with mesotheliomas that recur.</p><div id="CDR0000799420_rl_1075"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799420_rl_1075_1">Nagata S, Nakanishi R: Malignant pleural mesothelioma with cavity formation in a 16-year-old boy. Chest 127 (2): 655-7, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15706010" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15706010</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_2">Rosas-Salazar C, Gunawardena SW, Spahr JE: Malignant pleural mesothelioma in a child with ataxia-telangiectasia. Pediatr Pulmonol 48 (1): 94-7, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/22511568" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22511568</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_3">Orbach D, André N, Brecht IB, et al.: Mesothelioma in children and adolescents: the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) contribution. Eur J Cancer 140: 63-70, 2020. [<a href="https://pubmed.ncbi.nlm.nih.gov/33049597" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33049597</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_4">Tsao AS, Wistuba I, Roth JA, et al.: Malignant pleural mesothelioma. J Clin Oncol 27 (12): 2081-90, 2009. [<a href="/pmc/articles/PMC4881753/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4881753</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/19255316" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19255316</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_5">Carbone M, Ferris LK, Baumann F, et al.: BAP1 cancer syndrome: malignant mesothelioma, uveal and cutaneous melanoma, and MBAITs. J Transl Med 10: 179, 2012. [<a href="/pmc/articles/PMC3493315/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3493315</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/22935333" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22935333</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_6">Janes SM, Alrifai D, Fennell DA: Perspectives on the Treatment of Malignant Pleural Mesothelioma. N Engl J Med 385 (13): 1207-1218, 2021. [<a href="https://pubmed.ncbi.nlm.nih.gov/34551230" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 34551230</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_7">Hofmann J, Mintzer D, Warhol MJ: Malignant mesothelioma following radiation therapy. Am J Med 97 (4): 379-82, 1994. [<a href="https://pubmed.ncbi.nlm.nih.gov/7942942" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 7942942</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_8">Pappo AS, Santana VM, Furman WL, et al.: Post-irradiation malignant mesothelioma. Cancer 79 (1): 192-3, 1997. [<a href="https://pubmed.ncbi.nlm.nih.gov/8988749" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 8988749</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_9">Kelsey A: Mesothelioma in childhood. Pediatr Hematol Oncol 11 (5): 461-2, 1994 Sep-Oct. [<a href="https://pubmed.ncbi.nlm.nih.gov/7826842" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 7826842</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_10">Moran CA, Albores-Saavedra J, Suster S: Primary peritoneal mesotheliomas in children: a clinicopathological and immunohistochemical study of eight cases. Histopathology 52 (7): 824-30, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18494612" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18494612</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_11">Cioffredi LA, Jänne PA, Jackman DM: Treatment of peritoneal mesothelioma in pediatric patients. Pediatr Blood Cancer 52 (1): 127-9, 2009. [<a href="https://pubmed.ncbi.nlm.nih.gov/18819151" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18819151</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_12">Vermersch S, Arnaud A, Orbach D, et al.: Multicystic and diffuse malignant peritoneal mesothelioma in children. Pediatr Blood Cancer 67 (6): e28286, 2020. [<a href="https://pubmed.ncbi.nlm.nih.gov/32277799" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 32277799</span></a>]</div></li></ol></div></div><div id="CDR0000799420__2463"><h2 id="_CDR0000799420__2463_">Genomic Alterations</h2><p id="CDR0000799420__2311">Malignant mesotheliomas found in children, adolescents, and young adults are not often associated with asbestos exposures. This differs from most malignant mesotheliomas seen in adults. Recurring <i>ALK</i> gene fusions have been described in children and adolescents with mesothelioma. These fusions occur most often in female patients with peritoneal primary mesotheliomas. <i>ALK</i> gene fusions involve various partner genes, including <i>STRN</i>, <i>TPM1</i>, and <i>EML4</i>.[<a class="bk_pop" href="#CDR0000799420_rl_2463_1">1</a>]</p><div id="CDR0000799420_rl_2463"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799420_rl_2463_1">Argani P, Lian DWQ, Agaimy A, et al.: Pediatric Mesothelioma With ALK Fusions: A Molecular and Pathologic Study of 5 Cases. Am J Surg Pathol 45 (5): 653-661, 2021. [<a href="/pmc/articles/PMC8035308/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC8035308</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/33399341" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33399341</span></a>]</div></li></ol></div></div><div id="CDR0000799420__1080"><h2 id="_CDR0000799420__1080_">Diagnostic Evaluation</h2><p id="CDR0000799420__1081">Diagnostic thoracoscopy should be considered in suspicious cases to confirm diagnosis.[<a class="bk_pop" href="#CDR0000799420_rl_1080_1">1</a>] Cross-sectional imaging may suggest the diagnosis of peritoneal mesothelioma, but diagnostic biopsy by laparoscopy or open laparotomy is required. </p><div id="CDR0000799420_rl_1080"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799420_rl_1080_1">Nagata S, Nakanishi R: Malignant pleural mesothelioma with cavity formation in a 16-year-old boy. Chest 127 (2): 655-7, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15706010" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15706010</span></a>]</div></li></ol></div></div><div id="CDR0000799420__1864"><h2 id="_CDR0000799420__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000799420__1865">Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[<a class="bk_pop" href="#CDR0000799420_rl_1864_1">1</a>] Referral to medical centers with multidisciplinary teams of cancer specialists experienced in treating cancers that occur in childhood and adolescence should be considered. This multidisciplinary team approach incorporates the skills
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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="CDR0000799420__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="CDR0000799420__1867">For information about supportive care for children and adolescents with cancer, see the summaries on <a href="https://www.cancer.gov/publications/pdq/information-summaries/supportive-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Supportive and Palliative Care</a>.</p><p id="CDR0000799420__1868">The American Academy of Pediatrics has outlined guidelines for
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pediatric cancer centers and their role in the treatment of pediatric patients
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with cancer.[<a class="bk_pop" href="#CDR0000799420_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 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 current standard therapy. 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="CDR0000799420__1869">Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[<a class="bk_pop" href="#CDR0000799420_rl_1864_3">3</a>-<a class="bk_pop" href="#CDR0000799420_rl_1864_5">5</a>] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. For information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors, see <a href="/books/n/pdqcis/CDR0000343584/">Late Effects of Treatment for Childhood Cancer</a>.</p><p id="CDR0000799420__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="#CDR0000799420_rl_1864_6">6</a>] The U.S. <a href="https://www.congress.gov/107/plaws/publ280/PLAW-107publ280.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 people. Therefore, all pediatric cancers are considered rare.</p><p id="CDR0000799420__1888">The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[<a class="bk_pop" href="#CDR0000799420_rl_1864_7">7</a>,<a class="bk_pop" href="#CDR0000799420_rl_1864_8">8</a>] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000799420__1871"><li class="half_rhythm"><div class="half_rhythm">A consensus effort between the European Union Joint Action on Rare Cancers and the European Cooperative Study Group for Rare Pediatric Cancers estimated that 11% of all cancers in patients younger than 20 years could be categorized as very rare. This consensus group defined very rare cancers as those with annual incidences of fewer than 2 cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than 2 cases per 1 million people were also included in the very rare group because there is a lack of knowledge and expertise in the management of these tumors.[<a class="bk_pop" href="#CDR0000799420_rl_1864_9">9</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">The Children's Oncology Group (COG) defines rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancers, melanomas and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinomas, nasopharyngeal carcinomas, and most adult-type carcinomas such as breast cancers, colorectal cancers, etc.).[<a class="bk_pop" href="#CDR0000799420_rl_1864_10">10</a>] These diagnoses account for about 5% of the cancers diagnosed in children aged 0 to 14 years and about 27% of the cancers diagnosed in adolescents aged 15 to 19 years.[<a class="bk_pop" href="#CDR0000799420_rl_1864_4">4</a>]</div><div class="half_rhythm"> Most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers in children aged 0 to 14 years and 9.3% of the cancers in adolescents aged 15 to 19 years.</div></li></ul><p id="CDR0000799420__1872">These rare cancers are extremely challenging to study because of the low number of patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the lack of clinical trials for adolescents with rare cancers.</p><p id="CDR0000799420__1837">Information about these tumors may also be found in sources relevant to
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adults with cancer, such as <a href="/books/n/pdqcis/CDR0000062895/">Malignant Mesothelioma Treatment</a>.</p><div id="CDR0000799420_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799420_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="CDR0000799420_rl_1864_2">American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. <a href="https://pediatrics.aappublications.org/content/134/2/410" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Also available online</a>. Last accessed December 15, 2023.</div></li><li><div class="bk_ref" id="CDR0000799420_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="CDR0000799420_rl_1864_4">National Cancer Institute: NCCR*Explorer: An interactive website for NCCR cancer statistics. Bethesda, MD: National Cancer Institute. <a href="https://NCCRExplorer.ccdi.cancer.gov/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online</a>. Last accessed December 15, 2023.</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1864_5">Surveillance Research Program, National Cancer Institute: SEER*Explorer: An interactive website for SEER cancer statistics. Bethesda, MD: National Cancer Institute. <a href="https://seer.cancer.gov/statistics-network/explorer/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online</a>. Last accessed August 18, 2023.</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1864_6">Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr. [<a href="https://pubmed.ncbi.nlm.nih.gov/24488779" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24488779</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1864_7">Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28687376" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28687376</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1864_8">DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28542893" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28542893</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1864_9">Ferrari A, Brecht IB, Gatta G, et al.: Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 110: 120-126, 2019. [<a href="https://pubmed.ncbi.nlm.nih.gov/30785015" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30785015</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1864_10">Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010. [<a href="/pmc/articles/PMC3020699/" ref="pagearea=cite-ref&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></ol></div></div><div id="CDR0000799420__1082"><h2 id="_CDR0000799420__1082_">Treatment of Childhood Mesothelioma</h2><p id="CDR0000799420__2305">Treatment options for pediatric patients with malignant pleural mesotheliomas are controversial. Outcomes are often poor in these individuals despite treatment with radical surgical resection, chemotherapy, and radiation therapy. Treatments that use newer chemotherapy agents and immunotherapies are under investigation.[<a class="bk_pop" href="#CDR0000799420_rl_1082_1">1</a>]</p><p id="CDR0000799420__2130">Treatment options for childhood malignant mesothelioma include the following:</p><ol id="CDR0000799420__2295"><li class="half_rhythm"><div><a href="#CDR0000799420__2464">Surgery</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000799420__2465">Chemotherapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000799420__2467">Surgery and hyperthermic compartmental chemotherapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000799420__2468">Radiation therapy</a>.</div></li><li class="half_rhythm"><div><a href="#CDR0000799420__2469">Targeted therapy (ceritinib)</a>.</div></li></ol><div id="CDR0000799420__2464"><h3>Surgery</h3><p id="CDR0000799420__1083">Radical surgical resection has been attempted in patients with mesotheliomas with mixed results.[<a class="bk_pop" href="#CDR0000799420_rl_1082_2">2</a>] In adults, durable responses may be achieved with multimodal therapy that includes extrapleural pneumonectomy and radiation therapy after combination chemotherapy with pemetrexed-cisplatin.[<a class="bk_pop" href="#CDR0000799420_rl_1082_3">3</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000810033/" class="def">Level of evidence B4</a>] However, this approach remains highly controversial.[<a class="bk_pop" href="#CDR0000799420_rl_1082_4">4</a>]</p></div><div id="CDR0000799420__2465"><h3>Chemotherapy</h3><p id="CDR0000799420__2306">The European Cooperative Study Group on Pediatric Rare Tumors retrospectively reviewed children, adolescents, and young adults (aged ≤21 years) with mesotheliomas who were treated between 1987 and 2018.[<a class="bk_pop" href="#CDR0000799420_rl_1082_5">5</a>] Investigators identified 15 male patients and 18 female patients with mesotheliomas. Only one patient had a documented asbestos exposure. In most patients, the primary tumor was located in the peritoneum (23 patients). Tumor histologies were either multicystic mesothelioma of the peritoneum (6 patients) or malignant mesothelioma (27 patients). </p><ul id="CDR0000799420__2466"><li class="half_rhythm"><div>The response rate to treatment with cisplatin-pemetrexed was 50% (6 of 12 cases). </div></li><li class="half_rhythm"><div>After a median follow-up period of 6.7 years (range, 0–20 years), the 5-year overall survival rate was 82.3%, and the event-free survival rate was 45.1%. </div></li><li class="half_rhythm"><div>All patients with multicystic mesothelioma remained alive after either surgery (5 patients) or cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (1 patient).</div></li></ul></div><div id="CDR0000799420__2467"><h3>Surgery and Hyperthermic Compartmental Chemotherapy</h3><p id="CDR0000799420__2121">Hyperthermic intrapleural/intraperitoneal chemotherapy (HIPEC) has been used to treat pleural and intraperitoneal mesotheliomas. HIPEC, in conjunction with radical surgical resection, has been used to treat adults with pleural mesotheliomas. Although results have been encouraging, HIPEC has not been validated in controlled clinical trials because pleural mesotheliomas are rare.[<a class="bk_pop" href="#CDR0000799420_rl_1082_1">1</a>,<a class="bk_pop" href="#CDR0000799420_rl_1082_6">6</a>,<a class="bk_pop" href="#CDR0000799420_rl_1082_7">7</a>] A single-institution study followed seven children with intraperitoneal mesotheliomas who were treated with surgery and HIPEC.[<a class="bk_pop" href="#CDR0000799420_rl_1082_8">8</a>] At last available follow-up, five of the seven patients were alive and had either minimal disease or no evaluable disease.</p></div><div id="CDR0000799420__2468"><h3>Radiation Therapy</h3><p id="CDR0000799420__1402">Pain is an infrequent symptom in patients with mesotheliomas. However, if pain occurs, radiation therapy may be
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used for palliation.
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</p></div><div id="CDR0000799420__2469"><h3>Targeted Therapy (Ceritinib)</h3><p id="CDR0000799420__2321">In one case report, a 13-year-old patient with a peritoneal mesothelioma and a <i>STRN</i>::<i>ALK</i> fusion responded to ceritinib treatment.[<a class="bk_pop" href="#CDR0000799420_rl_1082_9">9</a>]</p><p id="CDR0000799420__2299">For more information, see <a href="/books/n/pdqcis/CDR0000062895/">Malignant Mesothelioma Treatment</a>.
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</p></div><div id="CDR0000799420_rl_1082"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799420_rl_1082_1">Carbone M, Adusumilli PS, Alexander HR, et al.: Mesothelioma: Scientific clues for prevention, diagnosis, and therapy. CA Cancer J Clin 69 (5): 402-429, 2019. [<a href="/pmc/articles/PMC8192079/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC8192079</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/31283845" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 31283845</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_2">Maziak DE, Gagliardi A, Haynes AE, et al.: Surgical management of malignant pleural mesothelioma: a systematic review and evidence summary. Lung Cancer 48 (2): 157-69, 2005. [<a href="https://pubmed.ncbi.nlm.nih.gov/15829316" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15829316</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_3">Krug LM, Pass HI, Rusch VW, et al.: Multicenter phase II trial of neoadjuvant pemetrexed plus cisplatin followed by extrapleural pneumonectomy and radiation for malignant pleural mesothelioma. J Clin Oncol 27 (18): 3007-13, 2009. [<a href="/pmc/articles/PMC3646305/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3646305</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/19364962" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19364962</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_4">Treasure T: What is the best approach for surgery of malignant pleural mesothelioma? It is to put our efforts into obtaining trustworthy evidence for practice. J Thorac Cardiovasc Surg 151 (2): 307-9, 2016. [<a href="https://pubmed.ncbi.nlm.nih.gov/26519246" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26519246</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_5">Orbach D, André N, Brecht IB, et al.: Mesothelioma in children and adolescents: the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) contribution. Eur J Cancer 140: 63-70, 2020. [<a href="https://pubmed.ncbi.nlm.nih.gov/33049597" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 33049597</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_6">Nguyen D, Sugarbaker DJ, Burt BM: Therapeutic R2 resection for pleural mesothelioma. J Thorac Cardiovasc Surg 155 (6): 2734-2735, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/29477257" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29477257</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_7">Wald O, Sugarbaker DJ: New Concepts in the Treatment of Malignant Pleural Mesothelioma. Annu Rev Med 69: 365-377, 2018. [<a href="https://pubmed.ncbi.nlm.nih.gov/29029582" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29029582</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_8">Malekzadeh P, Good M, Hughes MS: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) with cisplatin in pediatric patients with peritoneal mesothelioma: a single institution experience and long term follow up. Int J Hyperthermia 38 (1): 326-331, 2021. [<a href="https://pubmed.ncbi.nlm.nih.gov/34139940" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 34139940</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_9">Rüschoff JH, Gradhand E, Kahraman A, et al.: STRN -ALK Rearranged Malignant Peritoneal Mesothelioma With Dramatic Response Following Ceritinib Treatment. JCO Precis Oncol 3: , 2019. [<a href="/pmc/articles/PMC7446511/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC7446511</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/32914035" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 32914035</span></a>]</div></li></ol></div></div><div id="CDR0000799420__1930"><h2 id="_CDR0000799420__1930_">Treatment Options Under Clinical Evaluation for Childhood Mesothelioma</h2><p id="CDR0000799420__1931">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, see the <a href="https://clinicaltrials.gov/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">ClinicalTrials.gov website</a>.</p><p id="CDR0000799420__1932">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000799420__1933"><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 in a patient's tumor (refractory or recurrent). Children and adolescents aged 1 to 21 years are eligible for the trial.</div><div class="half_rhythm">Patients with tumors that have molecular variants addressed by open treatment arms in the trial may be enrolled in treatment on Pediatric MATCH. Additional information can be obtained on the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials/nci-supported/pediatric-match" ref="pagearea=body&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="CDR0000799420__2296"><h2 id="_CDR0000799420__2296_">Latest Updates to This Summary (05/19/2023)</h2><p id="CDR0000799420__2297">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="CDR0000799420__2471">Editorial changes were made to this summary.</p><p id="CDR0000799420__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="#CDR0000799420__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® Cancer Information for Health Professionals</a> pages.
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</p></div><div id="CDR0000799420__AboutThis_1"><h2 id="_CDR0000799420__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000799420__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000799420__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood mesothelioma. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.</p></div><div id="CDR0000799420__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000799420__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="CDR0000799420__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000799420__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="CDR0000799420__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 Mesothelioma Treatment are:</p><ul><li class="half_rhythm"><div>Denise Adams, MD (Children's Hospital Boston)</div></li><li class="half_rhythm"><div>Karen J. Marcus, MD, FACR (Dana-Farber Cancer Institute/Boston Children's Hospital)</div></li><li class="half_rhythm"><div>William H. Meyer, MD</div></li><li class="half_rhythm"><div>Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)</div></li><li class="half_rhythm"><div>Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)</div></li><li class="half_rhythm"><div>Alberto S. Pappo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)</div></li><li class="half_rhythm"><div>Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)</div></li><li class="half_rhythm"><div>Stephen J. Shochat, MD (St. Jude Children's Research Hospital)</div></li></ul><p id="CDR0000799420__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="CDR0000799420__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000799420__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="CDR0000799420__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000799420__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="CDR0000799420__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000799420__AboutThis_15">PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Mesothelioma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/mesothelioma/hp/child-mesothelioma-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/mesothelioma/hp/child-mesothelioma-treatment-pdq</a>. Accessed <MM/DD/YYYY>. [PMID: 31593397]</p><p id="CDR0000799420__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="CDR0000799420__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000799420__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="CDR0000799420__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000799420__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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<div xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance"></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Views</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="PDF_download" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="/books/NBK547409.10/?report=reader">PubReader</a></li><li><a href="/books/NBK547409.10/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK547409" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK547409" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Pediatric Treatment Editorial Board. Childhood Mesothelioma Treatment (PDQ®): Health Professional Version. 2023 May 19. In: PDQ Cancer Information Summaries [Internet]. 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