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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="#__NBK547408_dtls__">Show details</a><div style="display:none" class="ui-widget" id="__NBK547408_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="_NBK547408_"><span class="title" itemprop="name">Childhood Pleuropulmonary Blastoma 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: September 17, 2021.</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="CDR0000799361__1556">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood pleuropulmonary blastoma. 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="CDR0000799361__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="CDR0000799361__1030"><h2 id="_CDR0000799361__1030_">Types of Pleuropulmonary Blastoma and Other <i>DICER1</i>-Associated Neoplasms</h2><p id="CDR0000799361__1031">Pleuropulmonary blastoma is a rare and highly aggressive pulmonary malignancy
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that can present as a pulmonary or pleural mass. In most cases, pleuropulmonary blastoma is associated with germline mutations of the <i>DICER1</i> gene. The <a href="https://www.ppbregistry.org/" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">International Pleuropulmonary Blastoma Registry</a> is a valuable resource for information on this rare malignancy.[<a class="bk_pop" href="#CDR0000799361_rl_1030_1">1</a>,<a class="bk_pop" href="#CDR0000799361_rl_1030_2">2</a>]</p><p id="CDR0000799361__1218">The following three subtypes of pleuropulmonary blastoma have been identified:</p><ul id="CDR0000799361__1032"><li class="half_rhythm"><div class="half_rhythm"><b>Type I:</b> A purely lung cystic neoplasm with subtle malignant changes that typically occurs in the first 2 years of life and has a good prognosis. The median age at diagnosis for Type I tumors is 8 months, and there is a slight male predominance. Transition from Type I to Type III occurs; however, a significant proportion of Type I lesions may not progress to Type II and Type III tumors.[<a class="bk_pop" href="#CDR0000799361_rl_1030_2">2</a>,<a class="bk_pop" href="#CDR0000799361_rl_1030_3">3</a>]</div><div class="half_rhythm">Histologically, these tumors appear as a multilocular cyst with variable numbers of primitive mesenchymal cells beneath a benign epithelial surface, with skeletal differentiation in one-half of the cases.[<a class="bk_pop" href="#CDR0000799361_rl_1030_3">3</a>] This form of disease can be clinically and pathologically deceptive because of its resemblance to some developmental lung cysts.</div></li><li class="half_rhythm"><div class="half_rhythm"><b>Type Ir:</b> A purely cystic tumor that lacks a primitive cell component. The <i>r</i> designation signifies regression or nonprogression. Type Ir was originally recognized in older siblings of pleuropulmonary blastoma patients but can be seen in very young children. A lung cyst in an older individual with a <i>DICER1</i> mutation or in a relative of a pleuropulmonary blastoma patient is most likely to be Type Ir.[<a class="bk_pop" href="#CDR0000799361_rl_1030_2">2</a>]</div><div class="half_rhythm">In the Pleuropulmonary Blastoma Registry experience, most Type I and Ir cysts are unilateral (74%), half are unifocal, and 55% are larger than 5 cm. Pneumothorax may be present at diagnosis in up to 30% of Type I and Ir pleuropulmonary blastoma cases.[<a class="bk_pop" href="#CDR0000799361_rl_1030_2">2</a>]</div></li><li class="half_rhythm"><div class="half_rhythm"><b>Type II:</b> Type II exhibits both cystic and solid components. The solid areas have mixed blastomatous and sarcomatous features. Most of the cases exhibit rhabdomyoblasts, and nodules with cartilaginous differentiation are common.[<a class="bk_pop" href="#CDR0000799361_rl_1030_4">4</a>]</div><div class="half_rhythm">Anaplasia is present in up to 60% of the cases.[<a class="bk_pop" href="#CDR0000799361_rl_1030_5">5</a>] In the Pleuropulmonary Blastoma Registry, the median age at diagnosis was 35 months, and distant metastases were present at the time of diagnosis in 7% of patients.[<a class="bk_pop" href="#CDR0000799361_rl_1030_2">2</a>]</div></li><li class="half_rhythm"><div class="half_rhythm"><b>Type III:</b> A purely solid neoplasm, with the blastomatous and sarcomatous elements described above, and the presence of anaplasia in 70% of patients.[<a class="bk_pop" href="#CDR0000799361_rl_1030_5">5</a>-<a class="bk_pop" href="#CDR0000799361_rl_1030_7">7</a>]</div><div class="half_rhythm">Median age at diagnosis in the Pleuropulmonary Blastoma Registry was 41 months, and distant metastases were present at the time of diagnosis in 10% of patients.[<a class="bk_pop" href="#CDR0000799361_rl_1030_2">2</a>]</div></li></ul><p id="CDR0000799361__1495">The Pleuropulmonary Blastoma Registry reported on 350 centrally reviewed and confirmed cases of pleuropulmonary blastoma over a 50-year period (refer to <a class="figpopup" href="/books/NBK547408.3/table/CDR0000799361__1222/?report=objectonly" target="object" rid-figpopup="figCDR00007993611222" rid-ob="figobCDR00007993611222">Table 1</a>).[<a class="bk_pop" href="#CDR0000799361_rl_1030_2">2</a>] </p><div id="CDR0000799361__1222" class="table"><h3><span class="title">Table 1. Relative Proportions and Features of Pleuropulmonary Blastoma<sup>a</sup></span></h3><p class="large-table-link" style="display:none"><span class="right"><a href="/books/NBK547408.3/table/CDR0000799361__1222/?report=objectonly" target="object">View in own window</a></span></p><div class="large_tbl" id="__CDR0000799361__1222_lrgtbl__"><table class="no_margin"><thead><tr><th colspan="1" rowspan="1" style="vertical-align:top;"></th><th colspan="1" rowspan="1" style="vertical-align:top;">Type I </th><th colspan="1" rowspan="1" style="vertical-align:top;">Type Ir</th><th colspan="1" rowspan="1" style="vertical-align:top;">Type II</th><th colspan="1" rowspan="1" style="vertical-align:top;">Type II/III or III</th></tr></thead><tbody><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Relative proportion of pleuropulmonary blastoma cases
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</td><td colspan="2" rowspan="1" style="text-align:center;vertical-align:top;">33% </td><td colspan="1" rowspan="1" style="vertical-align:top;">35% </td><td colspan="1" rowspan="1" style="vertical-align:top;">32%</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Presence of germline <i>DICER1</i> mutation</td><td colspan="2" rowspan="1" style="text-align:center;vertical-align:top;">62%</td><td colspan="1" rowspan="1" style="vertical-align:top;">63%</td><td colspan="1" rowspan="1" style="vertical-align:top;">75%</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">Median age at diagnosis (months)</td><td colspan="1" rowspan="1" style="vertical-align:top;">8 </td><td colspan="1" rowspan="1" style="vertical-align:top;">47</td><td colspan="1" rowspan="1" style="vertical-align:top;">35 </td><td colspan="1" rowspan="1" style="vertical-align:top;">41</td></tr><tr><td colspan="1" rowspan="1" style="vertical-align:top;">5-year overall survival
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</td><td colspan="1" rowspan="1" style="vertical-align:top;">89% </td><td colspan="1" rowspan="1" style="vertical-align:top;">100%</td><td colspan="1" rowspan="1" style="vertical-align:top;">71% </td><td colspan="1" rowspan="1" style="vertical-align:top;">53%</td></tr></tbody></table></div><div><div><dl class="temp-labeled-list small"><dt></dt><dd><div><p class="no_margin"><sup>a</sup>Adapted from Messinger et al.[<a class="bk_pop" href="#CDR0000799361_rl_1030_2">2</a>]</p></div></dd></dl></div></div></div><div id="CDR0000799361_rl_1030"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799361_rl_1030_1">The International Pleuropulmonary Blastoma/DICER1 Registry. Minneapolis, Minn: Children's Hospitals and Clinics of Minnesota. <a href="https://www.ppbregistry.org/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online</a>. Last accessed January 31, 2020.</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1030_2">Messinger YH, Stewart DR, Priest JR, et al.: Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the International Pleuropulmonary Blastoma Registry. Cancer 121 (2): 276-85, 2015. [<a href="/pmc/articles/PMC4293209/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4293209</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/25209242" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25209242</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1030_3">Hill DA, Jarzembowski JA, Priest JR, et al.: Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the international pleuropulmonary blastoma registry. Am J Surg Pathol 32 (2): 282-95, 2008. [<a href="https://pubmed.ncbi.nlm.nih.gov/18223332" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 18223332</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1030_4">Priest JR, McDermott MB, Bhatia S, et al.: Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer 80 (1): 147-61, 1997. [<a href="https://pubmed.ncbi.nlm.nih.gov/9210721" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 9210721</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1030_5">Dehner LP, Messinger YH, Schultz KA, et al.: Pleuropulmonary Blastoma: Evolution of an Entity as an Entry into a Familial Tumor Predisposition Syndrome. Pediatr Dev Pathol 18 (6): 504-11, 2015 Nov-Dec. [<a href="/pmc/articles/PMC9743680/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC9743680</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/26698637" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 26698637</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1030_6">Priest JR, Hill DA, Williams GM, et al.: Type I pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry. J Clin Oncol 24 (27): 4492-8, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16983119" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16983119</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1030_7">Miniati DN, Chintagumpala M, Langston C, et al.: Prenatal presentation and outcome of children with pleuropulmonary blastoma. J Pediatr Surg 41 (1): 66-71, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/16410110" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16410110</span></a>]</div></li></ol></div></div><div id="CDR0000799361__2401"><h2 id="_CDR0000799361__2401_">Molecular Features</h2><p id="CDR0000799361__2402">In one report, 15 of 16 pleuropulmonary blastoma tumors were positive for <i>IGF1R</i> expression by immunohistochemistry.[<a class="bk_pop" href="#CDR0000799361_rl_2401_1">1</a>] Genomic profiling showed amplification of the <i>IGF1R</i> gene in 4 of 16 pleuropulmonary blastoma tumors. All of these gene-amplified tumors were Type III.</p><div id="CDR0000799361_rl_2401"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799361_rl_2401_1">Vokuhl C, de Leon-Escapini L, Leuschner I: Strong Expression and Amplification of IGF1R in Pleuropulmonary Blastomas. Pediatr Dev Pathol 20 (6): 475-481, 2017 Nov-Dec. [<a href="https://pubmed.ncbi.nlm.nih.gov/28382840" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28382840</span></a>]</div></li></ol></div></div><div id="CDR0000799361__1496"><h2 id="_CDR0000799361__1496_">Prognostic Factors</h2><p id="CDR0000799361__2108">In a comprehensive analysis of 350 patients reported by the Pleuropulmonary Blastoma Registry, only two prognostic factors were identified: the type of pleuropulmonary blastoma and the presence of metastatic disease at diagnosis.[<a class="bk_pop" href="#CDR0000799361_rl_1496_1">1</a>] (Refer to <a class="figpopup" href="/books/NBK547408.3/table/CDR0000799361__1222/?report=objectonly" target="object" rid-figpopup="figCDR00007993611222" rid-ob="figobCDR00007993611222">Table 1</a>.) In three additional small cohort series, the ability to perform a complete surgical resection was also identified as a prognostic factor.[<a class="bk_pop" href="#CDR0000799361_rl_1496_2">2</a>-<a class="bk_pop" href="#CDR0000799361_rl_1496_4">4</a>]</p><p id="CDR0000799361__2109">The presence of a germline <i>DICER1</i> mutation is not a prognostic factor.[<a class="bk_pop" href="#CDR0000799361_rl_1496_1">1</a>]</p><div id="CDR0000799361_rl_1496"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799361_rl_1496_1">Messinger YH, Stewart DR, Priest JR, et al.: Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the International Pleuropulmonary Blastoma Registry. Cancer 121 (2): 276-85, 2015. [<a href="/pmc/articles/PMC4293209/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4293209</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/25209242" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25209242</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1496_2">Indolfi P, Bisogno G, Casale F, et al.: Prognostic factors in pleuro-pulmonary blastoma. Pediatr Blood Cancer 48 (3): 318-23, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/16619221" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16619221</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1496_3">Bisogno G, Brennan B, Orbach D, et al.: Treatment and prognostic factors in pleuropulmonary blastoma: an EXPeRT report. Eur J Cancer 50 (1): 178-84, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/24041875" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24041875</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1496_4">Sparber-Sauer M, Seitz G, Kirsch S, et al.: The impact of local control in the treatment of type II/III pleuropulmonary blastoma. Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS). J Surg Oncol 115 (2): 164-172, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28103635" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28103635</span></a>]</div></li></ol></div></div><div id="CDR0000799361__1033"><h2 id="_CDR0000799361__1033_">Risk Factors</h2><p id="CDR0000799361__1034">Close to two-thirds of patients with pleuropulmonary blastoma have a germline <i>DICER1</i> mutation. Approximately one-third of families of children with pleuropulmonary blastoma manifest a number of dysplastic and/or neoplastic conditions comprising the DICER1 syndrome.[<a class="bk_pop" href="#CDR0000799361_rl_1033_1">1</a>-<a class="bk_pop" href="#CDR0000799361_rl_1033_3">3</a>]</p><p id="CDR0000799361__1351">Germline <i>DICER1</i> mutations have been associated with the following:[<a class="bk_pop" href="#CDR0000799361_rl_1033_1">1</a>-<a class="bk_pop" href="#CDR0000799361_rl_1033_5">5</a>]</p><ul id="CDR0000799361__2127"><li class="half_rhythm"><div>Cystic nephroma and Wilms tumor. Up to 10% of patients with pleuropulmonary blastoma have been reported to develop cystic nephroma or Wilms tumor, which are the most relevant associated malignancies. These tumors are also more prevalent among family members.[<a class="bk_pop" href="#CDR0000799361_rl_1033_6">6</a>]</div></li><li class="half_rhythm"><div>Ovarian sex cord–stromal tumors (especially Sertoli-Leydig cell tumor).</div></li><li class="half_rhythm"><div>Multinodular goiter.</div></li><li class="half_rhythm"><div>Uterine cervix embryonal rhabdomyosarcoma.</div></li><li class="half_rhythm"><div>Nasal chondromesenchymal hamartoma.</div></li><li class="half_rhythm"><div>Renal sarcoma.</div></li><li class="half_rhythm"><div>Pulmonary sequestration.</div></li><li class="half_rhythm"><div>Juvenile intestinal polyps.</div></li><li class="half_rhythm"><div>Ciliary body medulloepithelioma.</div></li><li class="half_rhythm"><div>Medulloblastoma.</div></li><li class="half_rhythm"><div>Pineoblastoma.</div></li><li class="half_rhythm"><div>Pituitary blastoma.</div></li><li class="half_rhythm"><div>Seminoma.</div></li></ul><p id="CDR0000799361__1352">The penetrance of <i>DICER1</i> germline mutations associated with each pathologic condition is not well understood, but lung cysts, pleuropulmonary blastoma, and thyroid nodules are the most commonly reported manifestations in individuals who have loss-of-function mutations.[<a class="bk_pop" href="#CDR0000799361_rl_1033_5">5</a>] Most associated conditions occur in children younger than 10 years, although ovarian tumors and multinodular goiters are described in children and adults aged up to 30 years.[<a class="bk_pop" href="#CDR0000799361_rl_1033_3">3</a>,<a class="bk_pop" href="#CDR0000799361_rl_1033_5">5</a>] A study of 102 individuals with <i>DICER1</i> germline mutations revealed a neoplasm risk of 5% by the age of 10 years and 19% by the age of 50 years.[<a class="bk_pop" href="#CDR0000799361_rl_1033_7">7</a>] Surveillance and screening recommendations have been proposed.[<a class="bk_pop" href="#CDR0000799361_rl_1033_5">5</a>]</p><div id="CDR0000799361_rl_1033"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799361_rl_1033_1">Hill DA, Ivanovich J, Priest JR, et al.: DICER1 mutations in familial pleuropulmonary blastoma. Science 325 (5943): 965, 2009. [<a href="/pmc/articles/PMC3098036/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3098036</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/19556464" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 19556464</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1033_2">Slade I, Bacchelli C, Davies H, et al.: DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome. J Med Genet 48 (4): 273-8, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21266384" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21266384</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1033_3">Foulkes WD, Bahubeshi A, Hamel N, et al.: Extending the phenotypes associated with DICER1 mutations. Hum Mutat 32 (12): 1381-4, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21882293" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21882293</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1033_4">Schultz KA, Pacheco MC, Yang J, et al.: Ovarian sex cord-stromal tumors, pleuropulmonary blastoma and DICER1 mutations: a report from the International Pleuropulmonary Blastoma Registry. Gynecol Oncol 122 (2): 246-50, 2011. [<a href="/pmc/articles/PMC3138876/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC3138876</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/21501861" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21501861</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1033_5">Schultz KAP, Williams GM, Kamihara J, et al.: DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies. Clin Cancer Res 24 (10): 2251-2261, 2018. [<a href="/pmc/articles/PMC6260592/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6260592</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/29343557" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29343557</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1033_6">Boman F, Hill DA, Williams GM, et al.: Familial association of pleuropulmonary blastoma with cystic nephroma and other renal tumors: a report from the International Pleuropulmonary Blastoma Registry. J Pediatr 149 (6): 850-854, 2006. [<a href="https://pubmed.ncbi.nlm.nih.gov/17137906" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 17137906</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1033_7">Stewart DR, Best AF, Williams GM, et al.: Neoplasm Risk Among Individuals With a Pathogenic Germline Variant in DICER1. J Clin Oncol 37 (8): 668-676, 2019. [<a href="/pmc/articles/PMC6553836/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6553836</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/30715996" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 30715996</span></a>]</div></li></ol></div></div><div id="CDR0000799361__2381"><h2 id="_CDR0000799361__2381_">Surveillance</h2><p id="CDR0000799361__2382">As with other cancer predisposition conditions, before individuals with <i>DICER1</i> mutations are screened, factors that must be considered include typical age of onset of each disease, potential benefits of early detection, and risks and availability of screening modalities. A consensus panel convened by the International Pleuropulmonary Blastoma Registry has proposed guidelines for surveillance. In addition to imaging-based surveillance, individuals and families can be counseled at each visit regarding potential signs and symptoms of <i>DICER1</i>-associated conditions and undergo appropriate age- and gender-specific preventive screening studies (refer to <a class="figpopup" href="/books/NBK547408.3/figure/CDR0000799361__2406/?report=objectonly" target="object" rid-figpopup="figCDR00007993612406" rid-ob="figobCDR00007993612406">Figure 1</a>).[<a class="bk_pop" href="#CDR0000799361_rl_2381_1">1</a>]</p><a id="CDR0000799361__2405"></a>
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<div id="CDR0000799361__2406" 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=574005_CDR0000799365.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/NBK547408.3/bin/CDR0000799365.jpg" alt="Table showing the suggested signs and symptoms and imaging surveillance by system for individuals with DICER1 pathogenic variants." class="tileshop" title="Click on image to zoom" /></a></div><div class="caption"><p>Figure 1. Suggested signs and symptoms and imaging surveillance by system for individuals with <i>DICER1</i> pathogenic variants. Adapted from Clinical Cancer Research, Copyright 2018, Volume 20/Issue 10, Pages 2251–2261, Kris Ann P. Schultz, Gretchen M. Williams, Junne Kamihara, et al.: DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies, with permission from AACR.</p></div></div>
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<div id="CDR0000799361_rl_2381"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799361_rl_2381_1">Schultz KAP, Williams GM, Kamihara J, et al.: DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies. Clin Cancer Res 24 (10): 2251-2261, 2018. [<a href="/pmc/articles/PMC6260592/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6260592</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/29343557" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29343557</span></a>]</div></li></ol></div></div><div id="CDR0000799361__1037"><h2 id="_CDR0000799361__1037_">Clinical Presentation</h2><p id="CDR0000799361__1038">Presenting symptoms are not specific. They commonly include the following: </p><ul id="CDR0000799361__1501"><li class="half_rhythm"><div>Respiratory distress.</div></li><li class="half_rhythm"><div>Fever.</div></li><li class="half_rhythm"><div>Chest pain. </div></li></ul><p id="CDR0000799361__1503">The tumor is usually located in the lung periphery, but it may be
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extrapulmonary with involvement of the heart/great vessels, mediastinum, diaphragm, and/or
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pleura.[<a class="bk_pop" href="#CDR0000799361_rl_1037_1">1</a>,<a class="bk_pop" href="#CDR0000799361_rl_1037_2">2</a>] Tumor embolism is a known risk, and radiographic evaluation of the central circulation is performed to identify potentially fatal embolic complications.[<a class="bk_pop" href="#CDR0000799361_rl_1037_3">3</a>]</p><div id="CDR0000799361_rl_1037"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799361_rl_1037_1">Indolfi P, Bisogno G, Casale F, et al.: Prognostic factors in pleuro-pulmonary blastoma. Pediatr Blood Cancer 48 (3): 318-23, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/16619221" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16619221</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1037_2">Bisogno G, Brennan B, Orbach D, et al.: Treatment and prognostic factors in pleuropulmonary blastoma: an EXPeRT report. Eur J Cancer 50 (1): 178-84, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/24041875" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24041875</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1037_3">Priest JR, Andic D, Arbuckle S, et al.: Great vessel/cardiac extension and tumor embolism in pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry. Pediatr Blood Cancer 56 (4): 604-9, 2011. [<a href="https://pubmed.ncbi.nlm.nih.gov/21298746" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21298746</span></a>]</div></li></ol></div></div><div id="CDR0000799361__1864"><h2 id="_CDR0000799361__1864_">Special Considerations for the Treatment of Children With Cancer</h2><p id="CDR0000799361__1865">Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[<a class="bk_pop" href="#CDR0000799361_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="CDR0000799361__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="CDR0000799361__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="CDR0000799361__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="#CDR0000799361_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="CDR0000799361__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="#CDR0000799361_rl_1864_3">3</a>] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy 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="CDR0000799361__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="#CDR0000799361_rl_1864_4">4</a>] The U.S. <a href="https://history.nih.gov/research/downloads/PL107-280.pdf" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">Rare Diseases Act of 2002</a> defines a rare disease as one that affects populations smaller than 200,000 people. Therefore, all pediatric cancers are considered rare.</p><p id="CDR0000799361__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="#CDR0000799361_rl_1864_5">5</a>,<a class="bk_pop" href="#CDR0000799361_rl_1864_6">6</a>] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:</p><ul id="CDR0000799361__1871"><li class="half_rhythm"><div class="half_rhythm">The Italian Tumori Rari in Eta Pediatrica group 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="#CDR0000799361_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="#CDR0000799361_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="#CDR0000799361_rl_1864_9">9</a>]</div><div class="half_rhythm">Most cancers within subgroup XI are either melanomas or thyroid cancer, with other 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="CDR0000799361__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="CDR0000799361__1837">Information about these tumors may also be found in sources relevant to
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adults with cancer.</p><div id="CDR0000799361_rl_1864"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799361_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="CDR0000799361_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 August 18, 2021.</div></li><li><div class="bk_ref" id="CDR0000799361_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="CDR0000799361_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="CDR0000799361_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="CDR0000799361_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="CDR0000799361_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="CDR0000799361_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="CDR0000799361_rl_1864_9">Howlader N, Noone AM, Krapcho M, et al., eds.: SEER Cancer Statistics Review, 1975-2012. 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 June 22, 2021.</div></li></ol></div></div><div id="CDR0000799361__1039"><h2 id="_CDR0000799361__1039_">Treatment of Childhood Pleuropulmonary Blastoma</h2><p id="CDR0000799361__1397">There are no standard treatment options for childhood pleuropulmonary blastoma. Current treatment regimens for these rare tumors have been informed by consensus opinion. </p><p id="CDR0000799361__2299">Treatment options for childhood pleuropulmonary blastoma include the following:</p><ol id="CDR0000799361__2300"><li class="half_rhythm"><div>Surgery.</div></li><li class="half_rhythm"><div>Adjuvant chemotherapy.</div></li></ol><p id="CDR0000799361__1040">A complete surgical resection is required for cure.[<a class="bk_pop" href="#CDR0000799361_rl_1039_1">1</a>]</p><p id="CDR0000799361__1041">Data from the International Pleuropulmonary Blastoma Registry and the European Cooperative Study Group in Pediatric Rare Tumors suggest that adjuvant chemotherapy may reduce the risk of recurrence.[<a class="bk_pop" href="#CDR0000799361_rl_1039_2">2</a>]; [<a class="bk_pop" href="#CDR0000799361_rl_1039_3">3</a>][<a href="/books/n/pdqcis/glossary_loe/def-item/glossary_loe_CDR0000335150/" class="def">Level of evidence: 3iiiA</a>] Responses to chemotherapy have been reported with agents
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similar to those used for the treatment of rhabdomyosarcoma.[<a class="bk_pop" href="#CDR0000799361_rl_1039_2">2</a>-<a class="bk_pop" href="#CDR0000799361_rl_1039_4">4</a>]</p><p id="CDR0000799361__1044">Some general treatment considerations from the International Pleuropulmonary Blastoma Registry include the following:[<a class="bk_pop" href="#CDR0000799361_rl_1039_2">2</a>,<a class="bk_pop" href="#CDR0000799361_rl_1039_5">5</a>]</p><ol id="CDR0000799361__1457"><li class="half_rhythm"><div><b>Type I and Type Ir:</b> Surgery is the treatment of choice. In the Pleuropulmonary Blastoma Registry series, the 5-year disease-free survival (DFS) and overall survival (OS) rates were 82% and 91%, respectively. Approximately 10% of cases may progress to Type II or Type III after surgery, but adjuvant chemotherapy does not appear to impact the rate of progression and survival.[<a class="bk_pop" href="#CDR0000799361_rl_1039_2">2</a>,<a class="bk_pop" href="#CDR0000799361_rl_1039_3">3</a>]</div></li><li class="half_rhythm"><div><b>Type II and Type III:</b> A multimodal sarcoma treatment approach is recommended, usually including rhabdomyosarcoma regimens and either upfront or delayed surgery.[<a class="bk_pop" href="#CDR0000799361_rl_1039_2">2</a>,<a class="bk_pop" href="#CDR0000799361_rl_1039_3">3</a>,<a class="bk_pop" href="#CDR0000799361_rl_1039_6">6</a>] Anthracycline-containing regimens appear to be superior.[<a class="bk_pop" href="#CDR0000799361_rl_1039_3">3</a>] The respective 5-year DFS and OS rates were 59% and 71% for Type II, and 37% and 53% for Type III.[<a class="bk_pop" href="#CDR0000799361_rl_1039_2">2</a>] The role of radiation therapy is not well defined. While the use of radiation did not impact survival in the International Pleuropulmonary Blastoma Registry series, only 20% of patients with Type II and Type III received it.[<a class="bk_pop" href="#CDR0000799361_rl_1039_2">2</a>] Approximately 50% of relapses occur in the brain.[<a class="bk_pop" href="#CDR0000799361_rl_1039_2">2</a>]</div></li></ol><div id="CDR0000799361_rl_1039"><h3>References</h3><ol><li><div class="bk_ref" id="CDR0000799361_rl_1039_1">Indolfi P, Bisogno G, Casale F, et al.: Prognostic factors in pleuro-pulmonary blastoma. Pediatr Blood Cancer 48 (3): 318-23, 2007. [<a href="https://pubmed.ncbi.nlm.nih.gov/16619221" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 16619221</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1039_2">Messinger YH, Stewart DR, Priest JR, et al.: Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the International Pleuropulmonary Blastoma Registry. Cancer 121 (2): 276-85, 2015. [<a href="/pmc/articles/PMC4293209/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC4293209</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/25209242" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 25209242</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1039_3">Bisogno G, Brennan B, Orbach D, et al.: Treatment and prognostic factors in pleuropulmonary blastoma: an EXPeRT report. Eur J Cancer 50 (1): 178-84, 2014. [<a href="https://pubmed.ncbi.nlm.nih.gov/24041875" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 24041875</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1039_4">Venkatramani R, Malogolowkin MH, Wang L, et al.: Pleuropulmonary blastoma: a single-institution experience. J Pediatr Hematol Oncol 34 (5): e182-5, 2012. [<a href="https://pubmed.ncbi.nlm.nih.gov/22584785" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 22584785</span></a>]</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1039_5">The International Pleuropulmonary Blastoma/DICER1 Registry. Minneapolis, Minn: Children's Hospitals and Clinics of Minnesota. <a href="https://www.ppbregistry.org/" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">Available online</a>. Last accessed January 31, 2020.</div></li><li><div class="bk_ref" id="CDR0000799361_rl_1039_6">Sparber-Sauer M, Seitz G, Kirsch S, et al.: The impact of local control in the treatment of type II/III pleuropulmonary blastoma. Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS). J Surg Oncol 115 (2): 164-172, 2017. [<a href="https://pubmed.ncbi.nlm.nih.gov/28103635" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 28103635</span></a>]</div></li></ol></div></div><div id="CDR0000799361__1603"><h2 id="_CDR0000799361__1603_">Treatment Options Under Clinical Evaluation for Childhood Pleuropulmonary Blastoma</h2><p id="CDR0000799361__1907">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="CDR0000799361__1908">The following is an example of a national and/or institutional clinical trial that is currently being conducted:</p><ul id="CDR0000799361__1605"><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="CDR0000799361__2403"><h2 id="_CDR0000799361__2403_">Changes to This Summary (09/17/2021)</h2><p id="CDR0000799361__124">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="CDR0000799361__2409">
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<b>
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<a href="#CDR0000799361__1030">Types of Pleuropulmonary Blastoma and Other <i>DICER1</i>-Associated Neoplasms</a>
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</b>
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</p><p id="CDR0000799361__2410">This section was renamed from Types of Pleuropulmonary Blastoma.</p><p id="CDR0000799361__2411">
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<b>
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<a href="#CDR0000799361__1033">Risk Factors</a>
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</b>
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</p><p id="CDR0000799361__2412">Added <a href="#CDR0000799361__1352">text</a> to state that a study of 102 individuals with <i>DICER1</i> germline mutations revealed a neoplasm risk of 5% by the age of 10 years and 19% by the age of 50 years (cited Stewart et al. as reference 7).</p><p id="CDR0000799361__2413">
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<b>
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<a href="#CDR0000799361__1864">Special Considerations for the Treatment of Children With Cancer</a>
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</b>
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</p><p id="CDR0000799361__2414">Added American Academy of Pediatrics as <a href="#CDR0000799361__1868">reference 2</a>.</p><p id="CDR0000799361__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="#CDR0000799361__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="CDR0000799361__AboutThis_1"><h2 id="_CDR0000799361__AboutThis_1_">About This PDQ Summary</h2><div id="CDR0000799361__AboutThis_2"><h3>Purpose of This Summary</h3><p id="CDR0000799361__AboutThis_3">This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood pleuropulmonary blastoma. 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="CDR0000799361__AboutThis_4"><h3>Reviewers and Updates</h3><p id="CDR0000799361__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="CDR0000799361__AboutThis_22"> Board members review recently published articles each month to determine whether an article should:</p><ul id="CDR0000799361__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="CDR0000799361__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 Pleuropulmonary Blastoma 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="CDR0000799361__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="CDR0000799361__AboutThis_10"><h3>Levels of Evidence</h3><p id="CDR0000799361__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="CDR0000799361__AboutThis_12"><h3>Permission to Use This Summary</h3><p id="CDR0000799361__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="CDR0000799361__AboutThis_14">The preferred citation for this PDQ summary is:</p><p id="CDR0000799361__AboutThis_15">PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Pleuropulmonary Blastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: <a href="https://www.cancer.gov/types/lung/hp/child-pleuropulmonary-blastoma-treatment-pdq" ref="pagearea=body&targetsite=external&targetcat=link&targettype=uri">https://www.cancer.gov/types/lung/hp/child-pleuropulmonary-blastoma-treatment-pdq</a>. Accessed <MM/DD/YYYY>. </p><p id="CDR0000799361__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="CDR0000799361__AboutThis_17"><h3>Disclaimer</h3><p id="CDR0000799361__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="CDR0000799361__AboutThis_20"><h3>Contact Us</h3><p id="CDR0000799361__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="figCDR00007993612406"><img alt="Image CDR0000799365" src-large="/books/NBK547408.3/bin/CDR0000799365.jpg" /></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/NBK547408.3/?report=reader">PubReader</a></li><li><a href="/books/NBK547408.3/?report=printable">Print View</a></li><li><a data-jig="ncbidialog" href="#_ncbi_dlg_citbx_NBK547408" data-jigconfig="width:400,modal:true">Cite this Page</a><div id="_ncbi_dlg_citbx_NBK547408" style="display:none" title="Cite this Page"><div class="bk_tt">PDQ Pediatric Treatment Editorial Board. Childhood Pleuropulmonary Blastoma Treatment (PDQ®): Health Professional Version. 2021 Sep 17. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. <span class="bk_cite_avail"></span></div></div></li><li><a href="#" class="toggle-glossary-link" title="Enable/disable links to the glossary">Disable Glossary Links</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Version History</span></h3></div><a name="Shutter" sid="1" href="#" class="portlet_shutter shutter_closed" title="Show/hide content" remembercollapsed="true" pgsec_name="version_history" id="Shutter"></a></div><div class="portlet_content" style="display: none;"><ul xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="simple-list"><li><span class="bk_col_itm"><a href="/books/NBK547408.13/">NBK547408.13</a></span> August 23, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK547408.12/">NBK547408.12</a></span> April 26, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK547408.11/">NBK547408.11</a></span> February 8, 2024</li><li><span class="bk_col_itm"><a href="/books/NBK547408.10/">NBK547408.10</a></span> December 15, 2023</li><li><span class="bk_col_itm"><a href="/books/NBK547408.9/">NBK547408.9</a></span> April 4, 2023</li><li><span class="bk_col_itm"><a href="/books/NBK547408.8/">NBK547408.8</a></span> December 8, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK547408.7/">NBK547408.7</a></span> June 8, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK547408.6/">NBK547408.6</a></span> March 16, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK547408.5/">NBK547408.5</a></span> March 1, 2022</li><li><span class="bk_col_itm"><a href="/books/NBK547408.4/">NBK547408.4</a></span> December 16, 2021</li><li><span class="bk_col_itm">NBK547408.3</span> September 17, 2021 (Displayed Version)</li><li><span class="bk_col_itm"><a href="/books/NBK547408.2/">NBK547408.2</a></span> January 31, 2020</li><li><span class="bk_col_itm"><a href="/books/NBK547408.1/">NBK547408.1</a></span> October 3, 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="#CDR0000799361__1030" ref="log$=inpage&link_id=inpage">Types of Pleuropulmonary Blastoma and Other <i>DICER1</i>-Associated Neoplasms</a></li><li><a href="#CDR0000799361__2401" ref="log$=inpage&link_id=inpage">Molecular Features</a></li><li><a href="#CDR0000799361__1496" ref="log$=inpage&link_id=inpage">Prognostic Factors</a></li><li><a href="#CDR0000799361__1033" ref="log$=inpage&link_id=inpage">Risk Factors</a></li><li><a href="#CDR0000799361__2381" ref="log$=inpage&link_id=inpage">Surveillance</a></li><li><a href="#CDR0000799361__1037" ref="log$=inpage&link_id=inpage">Clinical Presentation</a></li><li><a href="#CDR0000799361__1864" ref="log$=inpage&link_id=inpage">Special Considerations for the Treatment of Children With Cancer</a></li><li><a href="#CDR0000799361__1039" ref="log$=inpage&link_id=inpage">Treatment of Childhood Pleuropulmonary Blastoma</a></li><li><a href="#CDR0000799361__1603" ref="log$=inpage&link_id=inpage">Treatment Options Under Clinical Evaluation for Childhood Pleuropulmonary Blastoma</a></li><li><a href="#CDR0000799361__2403" ref="log$=inpage&link_id=inpage">Changes to This Summary (09/17/2021)</a></li><li><a href="#CDR0000799361__AboutThis_1" ref="log$=inpage&link_id=inpage">About This PDQ Summary</a></li></ul></div></div><div class="portlet"><div class="portlet_head"><div class="portlet_title"><h3><span>Related publications</span></h3></div><a name="Shutter" sid="1" href="#" 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class="brieflinkpop offscreen_noflow"><span xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="invert">Review</span> Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version.<div class="brieflinkpopdesc"><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="author">PDQ Pediatric Treatment Editorial Board. </em><em xmlns:np="http://ncbi.gov/portal/XSLT/namespace" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" class="cit">PDQ Cancer Information Summaries. 2002</em></div></div></li><li class="brieflinkpopper two_line"><a class="brieflinkpopperctrl" href="/pubmed/31593397" ref="ordinalpos=1&linkpos=2&log$=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 Mesothelioma Treatment (PDQ®): Health Professional Version.</a><span 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