{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,3]],"date-time":"2026-04-03T02:29:58Z","timestamp":1775183398108,"version":"3.50.1"},"reference-count":23,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2021,9,5]],"date-time":"2021-09-05T00:00:00Z","timestamp":1630800000000},"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":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Children with progressive (PD) or relapsed disease (RD) of pleuropulmonary blastoma (PPB) type II\/III are known to have a very poor outcome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A retrospective review of children registered in national and European databases and trials (2000\u20132018) with diagnosis of PPB type II\/III and PD or RD was performed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 35 patients with PPB were analysed: patients with PD (<jats:italic>n<\/jats:italic>\u00a0=\u00a09) and RD (<jats:italic>n<\/jats:italic>\u00a0=\u00a026). Patients experienced PD at the median age of 3.9 years [range, 0.5\u201317.8] despite surgery, chemotherapy (CHT, <jats:italic>n<\/jats:italic>\u00a0=\u00a09) and radiotherapy (RT, <jats:italic>n<\/jats:italic>\u00a0=\u00a01) with a median time to progression of 0.58 years [range, 0.02\u20131.27] from diagnosis. All of them died. Patients suffered from RD at the median age of 4.3 years [1.7\u201315.1], median delay to relapse 1.03 years [range, 0.03\u20132.95]. RD occurred locally (<jats:italic>n<\/jats:italic>\u00a0=\u00a012), combined (<jats:italic>n<\/jats:italic>\u00a0=\u00a01) and in metastatic sites (<jats:italic>n<\/jats:italic>\u00a0=\u00a013): central nervous system (<jats:italic>n<\/jats:italic>\u00a0=\u00a011) and unspecified site (<jats:italic>n<\/jats:italic>\u00a0=\u00a02). Patients were treated with salvage CHT (<jats:italic>n<\/jats:italic>\u00a0=\u00a020), surgery (<jats:italic>n<\/jats:italic>\u00a0=\u00a010)\u00a0\u00b1\u00a0RT (<jats:italic>n<\/jats:italic>\u00a0=\u00a010). After a median follow\u2010up of 4.2 years [range, 2.1\u201314.6], a second complete remission (CR) was achieved in nine out of 26 patients. Patients were alive in the second CR (<jats:italic>n<\/jats:italic>\u00a0=\u00a06), in the third CR (<jats:italic>n<\/jats:italic>\u00a0=\u00a01), in partial remission (<jats:italic>n<\/jats:italic>\u00a0=\u00a02) and lost of follow\u2010up (<jats:italic>n<\/jats:italic>\u00a0=\u00a01). Five\u2010year event\u2010free survival (EFS) and overall survival (OS) for patients with RD were both 37% (\u00b119, CI 95%). Local therapy (surgery, RT) had a favourable impact on OS (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.03 and 0.02, respectively).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Cure of patients with RD of PPB type II\/III with multimodal treatment is possible but rare. Progressive PPB is fatal and patients need new treatment options.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.29268","type":"journal-article","created":{"date-parts":[[2021,9,6]],"date-time":"2021-09-06T05:47:30Z","timestamp":1630907250000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Children with progressive and relapsed pleuropulmonary blastoma: A European collaborative analysis"],"prefix":"10.1002","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9551-2399","authenticated-orcid":false,"given":"Monika","family":"Sparber\u2010Sauer","sequence":"first","affiliation":[{"name":"Klinikum der Landeshauptstadt Stuttgart gKA\u00e4R, Olgahospital, Stuttgart Cancer Center Zentrum f\u00fcr Kinder\u2010, Jugend\u2010 und Frauenmedizin, P\u00e4diatrie 5 (P\u00e4diatrische Onkologie, H\u00e4matologie, Immunologie)  Stuttgart 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