{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,16]],"date-time":"2026-03-16T20:11:37Z","timestamp":1773691897248,"version":"3.50.1"},"reference-count":30,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,11,24]],"date-time":"2022-11-24T00:00:00Z","timestamp":1669248000000},"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":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Purpose<\/jats:title><jats:p>Pleuropulmonary blastoma (PPB) is the most common lung cancer of infancy and early childhood and is associated with germline <jats:italic>DICER1<\/jats:italic> variants. Type I and Ir PPB are cystic lesions treated surgically, with a subset of children with type I receiving chemotherapy. Type II and III are more aggressive lesions, treated with surgery, intensive chemotherapy and potentially radiation. We sought to assess health\u2010related quality of life (HRQoL) in children with PPB and known germline <jats:italic>DICER1<\/jats:italic> variants.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Children with a diagnosis of PPB or germline <jats:italic>DICER1<\/jats:italic> pathogenic variant without history of PPB or other <jats:italic>DICER1<\/jats:italic>\u2010related neoplasm (<jats:italic>DICER1<\/jats:italic>+ only) were enrolled in the International PPB\/<jats:italic>DICER1<\/jats:italic> Registry. Parent reports for participants aged 2\u201317\u00a0years for the PedsQL v.4 and PedsQL Multidimensional Fatigue Scale v.3 were collected. Fatigue, physical, and psychosocial function scores were compared.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Analysis included 84 participants (PPB type Ir\u00a0=\u00a020, type I\u00a0=\u00a015, type II\/III\u00a0=\u00a027, <jats:italic>DICER1<\/jats:italic>+ only\u00a0=\u00a022). Total fatigue scores of participants with type I and II\/III PPB were lower compared to <jats:italic>DICER1<\/jats:italic>+ only, with effect size larger in type II\/III (\u22120.82 vs. \u22120.40). Total psychosocial and physical functioning scores were lower in participants with type I and type II\/III PPB compared to <jats:italic>DICER1<\/jats:italic>+ only, with larger effects noted in type II\/III. Female sex was suggestive of worse HRQoL for both type I\/Ir and type II\/III cohorts.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These data demonstrate the importance of regular HRQoL assessment in patients with a history of PPB as well as the importance and feasibility of studying HRQoL in children with rare tumors.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.30077","type":"journal-article","created":{"date-parts":[[2022,11,25]],"date-time":"2022-11-25T05:49:50Z","timestamp":1669355390000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Health\u2010related quality of life in children and adolescents with pleuropulmonary blastoma: A report from the International PPB\/<i>DICER1<\/i> Registry"],"prefix":"10.1002","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1611-4577","authenticated-orcid":false,"given":"Alexander T.","family":"Nelson","sequence":"first","affiliation":[{"name":"International Pleuropulmonary Blastoma\/<i>DICER1<\/i> Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"International Ovarian and Testicular Stromal Tumor Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"Cancer and Blood Disorders Children's Minnesota  Minneapolis Minnesota USA"},{"name":"University of Minnesota Medical School  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Dybvik","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma\/<i>DICER1<\/i> Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"International Ovarian and Testicular Stromal Tumor Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"Cancer and Blood Disorders Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paige","family":"Mallinger","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma\/<i>DICER1<\/i> Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"International Ovarian and Testicular Stromal Tumor Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"Cancer and Blood Disorders Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shelly","family":"Weiss McQuaid","sequence":"additional","affiliation":[{"name":"Department of Genetics, Birth Defects &amp; Metabolism Ann &amp; Robert H. Lurie Children's Hospital of Chicago  Chicago Illinois USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2749-6136","authenticated-orcid":false,"given":"Dave","family":"Watson","sequence":"additional","affiliation":[{"name":"Research Institute Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura A.","family":"Harney","sequence":"additional","affiliation":[{"name":"Westat  Rockville Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8193-1488","authenticated-orcid":false,"given":"Douglas R.","family":"Stewart","sequence":"additional","affiliation":[{"name":"Clinical Genetics Branch Division of Cancer Epidemiology and Genetics National Cancer Institute  Rockville Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Louis P.","family":"Dehner","sequence":"additional","affiliation":[{"name":"Lauren V. Ackerman Laboratory of Surgical Pathology Department of Pathology and Immunology Washington University Medical Center  St. Louis Missouri USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7990-1802","authenticated-orcid":false,"given":"Yoav H.","family":"Messinger","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma\/<i>DICER1<\/i> Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"International Ovarian and Testicular Stromal Tumor Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"Cancer and Blood Disorders Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alicia S.","family":"Kunin\u2010Batson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Minnesota Medical School  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1788-5832","authenticated-orcid":false,"given":"Kris Ann P.","family":"Schultz","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma\/<i>DICER1<\/i> Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"International Ovarian and Testicular Stromal Tumor Registry Children's Minnesota  Minneapolis Minnesota USA"},{"name":"Cancer and Blood Disorders Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,11,24]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.29032"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.21.02925"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097\u20100142"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1174334"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.12688\/f1000research.6746.2"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamanetworkopen.2021.0112"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2018.78.4678"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2016\u20102954"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2016.83"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2017.100"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ygyno.2017.09.034"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1102903"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401\u2010019\u201001997\u2010y"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401\u2010014\u20101285\u2010z"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401\u2010014\u20101318\u20107"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379\u2010020\u20100516\u20101"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2017\u201002698"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379\u2010020\u20100458\u20107"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078\u20100432.Ccr\u201017\u20103089"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24706"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00520\u2010016\u20103253\u20108"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.10428"},{"key":"e_1_2_9_24_1","volume-title":"Statistical Power Analysis for the Behavioral Sciences","author":"Cohen J","year":"1988"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1093\/sleep\/31.2.271"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.27949"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13187\u2010018\u20101419\u20104"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1080\/08880010802016649"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.26149"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20923"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25037"}],"container-title":["Pediatric Blood &amp; 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