{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T13:13:30Z","timestamp":1793106810296,"version":"4.5.0"},"reference-count":54,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[2015,11,1]],"date-time":"2015-11-01T00:00:00Z","timestamp":1446336000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2015,11]]},"abstract":"<jats:p>Pleuropulmonary blastoma (PPB) is the most common primary malignant neoplasm of the lung in children. Like other solid dysontogenic neoplasms, this tumor typically presents before 7 years of age. The earliest manifestation is the presence of a lung cyst(s), which is usually recognized in the first year of life and is difficult to differentiate on the basis of imaging studies from nonneoplastic cysts of early childhood. From a multilocular cyst, PPB has the potential to progress to a high-grade multipatterned primitive sarcoma. More than 65% of all affected children have a heterozygous germline mutation in DICER1. The DICER1 PPB familial tumor predisposition syndrome is initially recognized in most cases on the basis of PPB alone but also by several other unique and characteristic extrapulmonary tumors, including pediatric cystic nephroma, nasal chondromesenchymal hamartoma, nodular lesions of the thyroid, embryonal rhabdomyosarcoma of the cervix, and ciliary body medulloepithelioma.<\/jats:p>","DOI":"10.2350\/15-10-1732-oa.1","type":"journal-article","created":{"date-parts":[[2015,12,23]],"date-time":"2015-12-23T16:36:16Z","timestamp":1450888576000},"page":"504-511","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":72,"title":["Pleuropulmonary Blastoma: Evolution of an Entity as an Entry into a Familial Tumor Predisposition Syndrome"],"prefix":"10.1177","volume":"18","author":[{"given":"Louis P.","family":"Dehner","sequence":"first","affiliation":[{"name":"International Pleuropulmonary Blastoma Registry, Children's Hospital and Clinics of Minnesota, Minneapolis, MN 55404, USA"},{"name":"Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish and St. Louis Children's Hospitals, Washington University Medical Center, St. Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoav H.","family":"Messinger","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma Registry, Children's Hospital and Clinics of Minnesota, Minneapolis, MN 55404, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kris Ann P.","family":"Schultz","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma Registry, Children's Hospital and Clinics of Minnesota, Minneapolis, MN 55404, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gretchen M.","family":"Williams","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma Registry, Children's Hospital and Clinics of Minnesota, Minneapolis, MN 55404, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn","family":"Wikenheiser-Brokamp","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma Registry, Children's Hospital and Clinics of Minnesota, Minneapolis, MN 55404, USA"},{"name":"Division of Pathology and Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, and Department of Pathology, University of Cincinnati College of Medicine, Cincinnati, OH 45267, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. Ashley","family":"Hill","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma Registry, Children's Hospital and Clinics of Minnesota, Minneapolis, MN 55404, USA"},{"name":"Division of Pathology, Children's National Medical Center, Washington, DC 20010, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2015,12,1]]},"reference":[{"key":"bibr1-15-10-1732-OA.1","first-page":"265","volume":"73","author":"Frances D","year":"1983","journal-title":"Curr Top Pathol"},{"key":"bibr2-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1002\/path.1700820119"},{"key":"bibr3-15-10-1732-OA.1","volume-title":"World Health Organization Classification of Tumours of the Lung, Pleura, Thymus and Heart","author":"Travis WD","year":"2015","edition":"4"},{"key":"bibr4-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gyobfe.2007.02.014"},{"key":"bibr5-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2005.10.074"},{"key":"bibr6-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19881015)62:8<1516::AID-CNCR2820620812>3.0.CO;2-3"},{"key":"bibr7-15-10-1732-OA.1","first-page":"3","volume":"1","author":"Schneider DT","year":"2012","journal-title":"Pediatr Oncol"},{"key":"bibr8-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0142(19970701)80:1<147::AID-CNCR20>3.0.CO;2-X"},{"key":"bibr9-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(96)70393-1"},{"key":"bibr10-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1174334"},{"key":"bibr11-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0510839103"},{"key":"bibr12-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.canlet.2004.07.037"},{"key":"bibr13-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1038\/nature10275"},{"key":"bibr14-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1005242"},{"key":"bibr15-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1002\/path.4500"},{"key":"bibr16-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.29032"},{"key":"bibr17-15-10-1732-OA.1","first-page":"467","volume-title":"Stocker and Dehner's Pediatric Pathology","author":"Stocker JT","year":"2011","edition":"3"},{"key":"bibr18-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200308000-00012"},{"key":"bibr19-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200404000-00017"},{"key":"bibr20-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3181484165"},{"key":"bibr21-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1038\/onc.2014.150"},{"key":"bibr22-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2007.08.012"},{"key":"bibr23-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000000382"},{"key":"bibr24-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/S1526-0542(04)90055-3"},{"key":"bibr25-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.earlhumdev.2014.09.014"},{"key":"bibr26-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(96)90345-0"},{"key":"bibr27-15-10-1732-OA.1","unstructured":"Hall NJ, Chiu PPL, Langer JC. Morbidity after elective resection and prenatally diagnosed asymptomatic congenital pulmonary airway malformations. Pediatr Pulmonol. In press."},{"key":"bibr28-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2010.02.067"},{"key":"bibr29-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0030-1267923"},{"key":"bibr30-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25233"},{"key":"bibr31-15-10-1732-OA.1","first-page":"171","volume":"14","author":"Kotecha S","year":"2013","journal-title":"Paediatr Resp Rev"},{"key":"bibr32-15-10-1732-OA.1","first-page":"169","volume":"14","author":"Delacourt C","year":"2013","journal-title":"Paediatr Resp Rev"},{"key":"bibr33-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1053\/j.sempedsurg.2014.09.007"},{"key":"bibr34-15-10-1732-OA.1","first-page":"214","volume":"4","author":"Brenneman M","year":"2015","journal-title":"FlOOOResearch"},{"key":"bibr35-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2006.08.068"},{"key":"bibr36-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2013.242"},{"key":"bibr37-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31804d43a4"},{"key":"bibr38-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1097\/PCR.0000000000000027"},{"key":"bibr39-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1102903"},{"key":"bibr40-15-10-1732-OA.1","unstructured":"Schultz KA, Harris A, Messinger Y, Ovarian Tumors related to intronic mutations in\n                      DICER1:\n                      a report from the International Ovarian and Testicular Stromal Tumor Registry. Fam Cancer. In press."},{"key":"bibr41-15-10-1732-OA.1","volume-title":"GeneReviews","author":"Doros L","year":"2015"},{"key":"bibr42-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.2010.189779"},{"key":"bibr43-15-10-1732-OA.1","doi-asserted-by":"crossref","first-page":"e48","DOI":"10.3928\/01913913-20140709-03","volume":"51","author":"Kramer GD","year":"2014","journal-title":"J Pediatr Ophthalmol Strabismus"},{"key":"bibr44-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21600"},{"key":"bibr45-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-014-1474-9"},{"key":"bibr46-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1186\/s40463-015-0077-3"},{"key":"bibr47-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-014-1285-z"},{"key":"bibr48-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2014-1016"},{"key":"bibr49-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2011.185"},{"key":"bibr50-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2015.01.020"},{"key":"bibr51-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1002\/mgg3.110"},{"key":"bibr52-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1002\/path.4135"},{"key":"bibr53-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.1038\/nrc3802"},{"key":"bibr54-15-10-1732-OA.1","doi-asserted-by":"publisher","DOI":"10.5858\/2008-132-1079-PAMLTI"}],"container-title":["Pediatric and Developmental Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.2350\/15-10-1732-OA.1","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.2350\/15-10-1732-OA.1","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.2350\/15-10-1732-OA.1","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T02:41:33Z","timestamp":1777603293000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.2350\/15-10-1732-OA.1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,11]]},"references-count":54,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2015,11]]}},"alternative-id":["10.2350\/15-10-1732-OA.1"],"URL":"https:\/\/doi.org\/10.2350\/15-10-1732-oa.1","relation":{},"ISSN":["1093-5266","1615-5742"],"issn-type":[{"value":"1093-5266","type":"print"},{"value":"1615-5742","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,11]]}}}