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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: June 8, 2020.</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 who care for cancer 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 childhood, 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__1272">Mesothelioma may develop after successful treatment of an
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earlier cancer, especially after treatment with radiation.[<a class="bk_pop" href="#CDR0000799420_rl_1075_3">3</a>,<a class="bk_pop" href="#CDR0000799420_rl_1075_4">4</a>] The amount of exposure required to develop cancer is
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unknown. In adults,
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these tumors have been associated with exposure to asbestos, which was used as
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building insulation.[<a class="bk_pop" href="#CDR0000799420_rl_1075_5">5</a>] There is no information about the risk for children exposed to
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asbestos.</p><p id="CDR0000799420__1077">This tumor 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_6">6</a>-<a class="bk_pop" href="#CDR0000799420_rl_1075_8">8</a>]; [<a class="bk_pop" href="#CDR0000799420_rl_1075_9">9</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335144/" class="def">Level of evidence: 3iiA</a>] These tumors can spread over the surface of organs,
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without invading far into the underlying tissue, and may spread to regional or
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distant lymph nodes. </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">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_4">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_5">Hyers TM, Ohar JM, Crim C: Clinical controversies in asbestos-induced lung diseases. Semin Diagn Pathol 9 (2): 97-101, 1992. [<a href="https://pubmed.ncbi.nlm.nih.gov/1609162" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 1609162</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1075_6">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_7">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_8">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_9">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__1078"><h2 id="_CDR0000799420__1078_">Prognosis</h2><p id="CDR0000799420__1079">Benign and malignant mesotheliomas cannot be differentiated using histologic
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criteria. A poor prognosis is associated with lesions that are diffuse and
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invasive and with those that recur. In general, the course of the disease is
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slow, and long-term survival is common. </p><p id="CDR0000799420__2300">A French retrospective review identified 14 cases of peritoneal mesothelioma in patients younger than 18 years. A variety of treatments were employed, including surgery, heated intraperitoneal chemotherapy, and systemic chemotherapy. At a median follow up of 7 years, 8 of 14 patients experienced recurrences or disease progression.[<a class="bk_pop" href="#CDR0000799420_rl_1078_1">1</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335144/" class="def">Level of evidence: 3iiA</a>]</p><div id="CDR0000799420_rl_1078"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799420_rl_1078_1">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__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>]</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__1082"><h2 id="_CDR0000799420__1082_">Treatment of Childhood Mesothelioma</h2><p id="CDR0000799420__2130">Treatment options for childhood malignant mesothelioma include the following:</p><ol id="CDR0000799420__2295"><li class="half_rhythm"><div>Surgery.</div></li><li class="half_rhythm"><div>Chemotherapy.</div></li><li class="half_rhythm"><div>Radiation therapy.</div></li></ol><p id="CDR0000799420__1083">Radical surgical resection has been attempted with mixed results.[<a class="bk_pop" href="#CDR0000799420_rl_1082_1">1</a>] In adults, a multimodal therapy including extrapleural pneumonectomy and radiation therapy after combination chemotherapy with pemetrexed-cisplatin may achieve durable responses.[<a class="bk_pop" href="#CDR0000799420_rl_1082_2">2</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335132/" class="def">Level of evidence: 2A</a>] However, this approach remains highly controversial.[<a class="bk_pop" href="#CDR0000799420_rl_1082_3">3</a>] In children, treatment with various
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chemotherapeutic agents used for carcinomas or sarcomas may result in partial
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responses.[<a class="bk_pop" href="#CDR0000799420_rl_1082_4">4</a>-<a class="bk_pop" href="#CDR0000799420_rl_1082_7">7</a>]</p><p id="CDR0000799420__2121">Hyperthermic chemotherapy has been used to treat adults with pleural mesothelioma.[<a class="bk_pop" href="#CDR0000799420_rl_1082_8">8</a>,<a class="bk_pop" href="#CDR0000799420_rl_1082_9">9</a>]</p><p id="CDR0000799420__1402">Pain is an infrequent symptom; however, if pain occurs, radiation therapy may be
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used for palliation.
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</p><p id="CDR0000799420__1084">Papillary serous carcinoma of the peritoneum may be mistaken for
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mesothelioma.[<a class="bk_pop" href="#CDR0000799420_rl_1082_10">10</a>] This tumor generally involves all surfaces lining the
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abdominal organs, including the surfaces of the ovary. Treatment includes
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surgical resection whenever possible and use of chemotherapy with agents such
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as cisplatin, carboplatin, and paclitaxel.</p><p id="CDR0000799420__2299">(Refer to the PDQ summary on <a href="/books/n/pdqcis/CDR0000062895/">Malignant Mesothelioma Treatment [Adult]</a> for more information.)
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</p><div id="CDR0000799420_rl_1082"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799420_rl_1082_1">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_2">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_3">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_4">Milano E, Pourroy B, Rome A, et al.: Efficacy of a combination of pemetrexed and multiple redo-surgery in an 11-year-old girl with a recurrent multifocal abdominal mesothelioma. Anticancer Drugs 17 (10): 1231-4, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/17075325" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17075325</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_5">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_1082_6">Sugalski A, Davis M, Prasannan L, et al.: Clinical, histologic, and genetic features of mesothelioma in a 7-year-old child. Pediatr Blood Cancer 60 (1): 146-8, 2013. [<a href="https://pubmed.ncbi.nlm.nih.gov/22961710" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22961710</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_7">Kobayashi S, Waragai T, Sano H, et al.: Malignant peritoneal mesothelioma in a child: chemotherapy with gemcitabine and platinum was effective for the disease unresponsive to other treatments. Anticancer Drugs 25 (9): 1102-5, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/25010395" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25010395</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1082_8">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_9">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_10">Wall JE, Mandrell BN, Jenkins JJ, et al.: Effectiveness of paclitaxel in treating papillary serous carcinoma of the peritoneum in an adolescent. Am J Obstet Gynecol 172 (3): 1049-52, 1995. [<a href="https://pubmed.ncbi.nlm.nih.gov/7892850" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 7892850</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, refer to 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 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><p id="CDR0000799420__828">(Refer to the PDQ summary on <a href="/books/n/pdqcis/CDR0000062895/">Malignant Mesothelioma Treatment [Adult]</a> for more information.)
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</p></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 of childhood cancer 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 for children and adolescents with cancer. This multidisciplinary team approach incorporates the skills
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of the following health care professionals and others to ensure that children receive treatment, supportive care, and rehabilitation
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that will achieve optimal survival and quality of life:</p><ul id="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"> (Refer to the PDQ <a href="https://www.cancer.gov/publications/pdq/information-summaries/supportive-care" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Supportive and Palliative Care</a> summaries for specific information about supportive care for children and adolescents with cancer.)</p><p id="CDR0000799420__1868">Guidelines for
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pediatric cancer centers and their role in the treatment of pediatric patients
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with cancer have been outlined by the American Academy of Pediatrics.[<a class="bk_pop" href="#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 in these trials is offered to most patients and their families. Clinical
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trials for children and adolescents diagnosed with cancer are generally
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designed to compare potentially better therapy with therapy that is currently
|
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accepted as standard. Most of the progress made in identifying curative
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therapy for childhood cancers has been achieved through clinical trials.
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Information about ongoing clinical trials is available from the <a href="https://www.cancer.gov/about-cancer/treatment/clinical-trials" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">NCI website</a>.</p><p id="CDR0000799420__1869">Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality decreased by more than 50%.[<a class="bk_pop" href="#CDR0000799420_rl_1864_3">3</a>] Childhood and adolescent cancer survivors require close monitoring because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ summary on <a href="/books/n/pdqcis/CDR0000343584/">Late Effects of Treatment for Childhood Cancer</a> for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)</p><p id="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_4">4</a>] The U.S. <a href="https://history.nih.gov/download/attachments/1016866/PL107-280.pdf" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Rare Diseases Act of 2002</a> defines a rare disease as one that affects populations smaller than 200,000 persons. Therefore, all pediatric cancers are considered rare.</p><p id="CDR0000799420__1881">The designation of a rare tumor is not uniform among pediatric and adult groups. Adult rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people, and they are estimated to account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[<a class="bk_pop" href="#CDR0000799420_rl_1864_5">5</a>,<a class="bk_pop" href="#CDR0000799420_rl_1864_6">6</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">The Italian cooperative project on rare pediatric tumors (Tumori Rari in Eta Pediatrica [TREP]) defines a pediatric rare tumor as one with an incidence of less than two cases per 1 million population per year and is not included in other clinical trials.[<a class="bk_pop" href="#CDR0000799420_rl_1864_7">7</a>]</div></li><li class="half_rhythm"><div class="half_rhythm">The Children's Oncology Group has opted to define rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancer, melanoma and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinoma, nasopharyngeal carcinoma, and most adult-type carcinomas such as breast cancer, colorectal cancer, etc.).[<a class="bk_pop" href="#CDR0000799420_rl_1864_8">8</a>] These diagnoses account for about 4% of cancers diagnosed in children aged 0 to 14 years, compared with about 20% of cancers diagnosed in adolescents aged 15 to 19 years.[<a class="bk_pop" href="#CDR0000799420_rl_1864_9">9</a>]</div><div class="half_rhythm">Most cancers within subgroup XI are either melanomas or thyroid cancer, with the remaining subgroup XI cancer types accounting for only 1.3% of cancers in children aged 0 to 14 years and 5.3% of cancers in adolescents aged 15 to 19 years.</div></li></ul><p id="CDR0000799420__1872">These rare cancers are extremely challenging to study because of the low incidence of patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the lack of clinical trials for adolescents with rare cancers.</p><p id="CDR0000799420__1829">Information about these tumors may also be found in sources relevant to
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adults with cancer such as the PDQ summary on <a href="/books/n/pdqcis/CDR0000062895/">Malignant Mesothelioma Treatment (Adult)</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">Corrigan JJ, Feig SA; American Academy of Pediatrics: Guidelines for pediatric cancer centers. Pediatrics 113 (6): 1833-5, 2004. [<a href="https://pubmed.ncbi.nlm.nih.gov/15173520" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 15173520</span></a>]</div></li><li><div class="bk_ref" id="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">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_5">Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28687376" ref="pagearea=cite-ref&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_6">DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28542893" ref="pagearea=cite-ref&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_7">Ferrari A, Bisogno G, De Salvo GL, et al.: The challenge of very rare tumours in childhood: the Italian TREP project. Eur J Cancer 43 (4): 654-9, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/17049226" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17049226</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1864_8">Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010. [<a href="/pmc/articles/PMC3020699/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3020699</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/20956621" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 20956621</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799420_rl_1864_9">Howlader N, Noone AM, Krapcho M, et al., eds.: SEER Cancer Statistics Review, 1975-2012. Bethesda, Md: National Cancer Institute, 2015. <a href="http://seer.cancer.gov/csr/1975_2012/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Also available online</a>. Last accessed October 05, 2020.</div></li></ol></div></div><div id="CDR0000799420__2296"><h2 id="_CDR0000799420__2296_">Changes to This Summary (06/08/2020)</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__2301"><b><a href="#CDR0000799420__1075">Incidence, Risk Factors, and Clinical Presentation</a></b></p><p id="CDR0000799420__2302">Added Vermersch et al. as <a href="#CDR0000799420__1077">reference 9</a> and level of evidence 3iiA.</p><p id="CDR0000799420__2303"><b><a href="#CDR0000799420__1078">Prognosis</a></b></p><p id="CDR0000799420__2304">Added <a href="#CDR0000799420__2300">text</a> to state that a French retrospective review identified 14 cases of peritoneal mesothelioma in patients younger than 18 years. A variety of treatments were employed, including surgery, heated intraperitoneal chemotherapy, and systemic chemotherapy. At a median follow up of 7 years, 8 of 14 patients experienced recurrences or disease progression (cited Vermersch et al. as reference 1 and level of evidence 3iiA).</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® - NCI's Comprehensive Cancer Database</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 who care for cancer 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>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>. </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/NBK547409.2/?report=reader">PubReader</a></li><li><a href="/books/NBK547409.2/?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. 2020 Jun 8. In: PDQ Cancer Information Summaries [Internet]. 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2019</li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>In this Page</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter" title="Show/hide content" remembercollapsed="true" pgsec_name="page-toc" id="Shutter"></a></div><div class="portlet_content"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><a href="#CDR0000799420__1075" ref="log$=inpage&link_id=inpage">Incidence, Risk Factors, and Clinical Presentation</a></li><li><a href="#CDR0000799420__1078" ref="log$=inpage&link_id=inpage">Prognosis</a></li><li><a href="#CDR0000799420__1080" ref="log$=inpage&link_id=inpage">Diagnostic Evaluation</a></li><li><a href="#CDR0000799420__1082" ref="log$=inpage&link_id=inpage">Treatment of Childhood Mesothelioma</a></li><li><a href="#CDR0000799420__1930" ref="log$=inpage&link_id=inpage">Treatment Options Under Clinical Evaluation for Childhood Mesothelioma</a></li><li><a href="#CDR0000799420__1864" ref="log$=inpage&link_id=inpage">Special Considerations for the Treatment of Children With Cancer</a></li><li><a href="#CDR0000799420__2296" ref="log$=inpage&link_id=inpage">Changes to This Summary (06/08/2020)</a></li><li><a href="#CDR0000799420__AboutThis_1" ref="log$=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/NBK547401/">Patient Version</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div 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Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/31909942" ref="ordinalpos=1&linkpos=2&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Pheochromocytoma and Paraganglioma Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Pheochromocytoma and Paraganglioma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em 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xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Craniopharyngioma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/38630971" ref="ordinalpos=1&linkpos=5&log$=relatedreviews&logdbfrom=pubmed"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Myelodysplastic Neoplasms Treatment (PDQ®): Health Professional Version.</a><span class="source">[PDQ Cancer Information Summari...]</span><div 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