{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T21:31:59Z","timestamp":1792877519160,"version":"4.5.0"},"reference-count":37,"publisher":"Springer Science and Business Media LLC","license":[{"start":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T00:00:00Z","timestamp":1776729600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T00:00:00Z","timestamp":1776729600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Universit\u00e4tsklinikum Leipzig"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Radiol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Pleuropulmonary blastoma is a rare pediatric lung tumor of mesenchymal origin, with prognosis varying by subtype. While types II and III are usually identifiable on imaging due to their solid tumor components, the purely cystic type I represents a particular diagnostic challenge. In imaging, it is often difficult to distinguish from benign congenital lung malformations such as congenital pulmonary airway malformation (CPAM, formerly congenital cystic adenomatoid malformation or CCAM), sequestration, or bronchogenic cyst. Accurate differentiation, however, is clinically relevant, as misclassification may lead to inadequate treatment or an increased risk of disease progression. This article highlights the role of imaging modalities (computed tomography, magnetic resonance imaging, including ultrashort echo time sequences), as well as histopathology and genetics\u2014especially\n                    <jats:italic>DICER1<\/jats:italic>\n                    mutation\u2014in the diagnosis and therapeutic decision-making of pleuropulmonary blastoma. A modified flowchart based on Feinberg et al. provides practical guidance. For patients with pleuropulmonary blastoma carrying a\n                    <jats:italic>DICER1<\/jats:italic>\n                    mutation, specific organ-targeted follow-up recommendations are provided to enable early detection of associated tumors.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Graphical Abstract<\/jats:bold>\n                  <\/jats:p>","DOI":"10.1007\/s00247-026-06611-4","type":"journal-article","created":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T11:54:12Z","timestamp":1776772452000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Pleuropulmonary blastoma and the challenge of distinguishing the cystic form (type I) from other congenital lung malformations"],"prefix":"10.1007","author":[{"given":"Franz Wolfgang","family":"Hirsch","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Gr\u00e4fe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ina","family":"Sorge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Lacher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maximilian","family":"von Laffert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,4,21]]},"reference":[{"key":"6611_CR1","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1016\/j.ejca.2018.12.031","volume":"110","author":"A Ferrari","year":"2019","unstructured":"Ferrari A, Brecht IB, Gatta G et al (2019) Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. 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