{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T18:21:51Z","timestamp":1790446911890,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,12,16]],"date-time":"2021-12-16T00:00:00Z","timestamp":1639612800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pleuropulmonary blastoma (PPB) is a rare pediatric tumor of the pleura and pulmonary mesenchyme, associated with pathogenic germline <jats:italic>DICER1<\/jats:italic> mutations. Although the most common site of metastasis is the central nervous system (CNS), patients with CNS metastasis have dismal outcome. We report a case of a patient presenting with type II PPB and intracranial and bone metastases. We describe a multimodal therapy approach and highlight the use of intraventricular topotecan for isolated CNS recurrence. In addition, a new pathogenic germline mutation heterozygous for the c.1234delT of <jats:italic>DICER1<\/jats:italic> was identified. Patient remains in remission 3\u00a0years after recurrence.<\/jats:p>","DOI":"10.1002\/pbc.29529","type":"journal-article","created":{"date-parts":[[2021,12,16]],"date-time":"2021-12-16T12:33:29Z","timestamp":1639658009000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Intraventricular topotecan in the successful treatment of recurrent CNS pleuropulmonary blastoma"],"prefix":"10.1002","volume":"69","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6717-2347","authenticated-orcid":false,"given":"Jenny","family":"Shim","sequence":"first","affiliation":[{"name":"Department of Pediatrics Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Aflac Cancer and Blood Disorders Center at the Children's Healthcare of Atlanta  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Reisner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Department of Neurosurgery, Pediatrics Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natia","family":"Esiashvili","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology Winship Cancer Institute of Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis","family":"Rapkin","sequence":"additional","affiliation":[{"name":"Department of Oncology Children's Hospital of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6691-5380","authenticated-orcid":false,"given":"Thomas","family":"Olson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Aflac Cancer and Blood Disorders Center at the Children's Healthcare of Atlanta  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,12,16]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19881015)62:8<1516::AID-CNCR2820620812>3.0.CO;2-3"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.29032"},{"issue":"3","key":"e_1_2_7_4_1","first-page":"206","article-title":"Pleuropulmonary blastoma: more than a lung neoplasm of childhood","volume":"116","author":"Dehner LP","year":"2019","journal-title":"Mo Med"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.3109\/15513815.2013.839011"},{"key":"e_1_2_7_6_1","article-title":"Pleuropulmonary blastoma: a case report and review of the literature","volume":"2014","author":"Khan AA","year":"2014","journal-title":"Case Rep Pathol"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2013.08.015"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20842"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20937"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.20928"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2019.06.009"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000000972"},{"issue":"8","key":"e_1_2_7_13_1","first-page":"86","article-title":"Current management of pleuropulmonary blastoma: a surgical perspective","volume":"6","author":"Knight S","year":"2019","journal-title":"Children (Basel)"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(20000915)89:6<1396::AID-CNCR25>3.0.CO;2-2"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.27628"},{"issue":"4","key":"e_1_2_7_16_1","first-page":"237","article-title":"Intraventricular topotecan for the treatment of neoplastic meningitis: five case studies","volume":"3","author":"Walker J","year":"2012","journal-title":"J Adv Pract Oncol"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1215\/15228517-2007-059"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24309"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-012-0182-9"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers10050143"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrc3802"}],"container-title":["Pediatric Blood &amp; 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