{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T22:10:19Z","timestamp":1792793419476,"version":"4.5.0"},"reference-count":43,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2021,2,20]],"date-time":"2021-02-20T00:00:00Z","timestamp":1613779200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center","id":[{"id":"https:\/\/ror.org\/05dq2gs74","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,7,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Mucinous adenocarcinoma arising in unresected congenital pulmonary airway malformation (CPAM) is rare. Underlying driver mutations in addition to KRAS gain-of-function mutations in this setting and the long-term outcomes of these patients are unknown.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We report a case of metastatic mucinous adenocarcinoma harboring both KRAS and GNAS mutations arising in a type 1 CPAM of a 14-year-old male. A literature review was performed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Next-generation sequencing revealed identical KRAS (G12V) mutations in both the CPAM and metastatic adenocarcinoma and a missense mutation in the GNAS (R201C) gene in the metastatic adenocarcinoma only. Median survival was 23 and 4 years for patients with localized (no or limited spread within the same lobe of CPAM) and distant involvement (spread to any different lobe of CPAM) of mucinous cells, respectively (95% confidence interval, 23-23 and 1.5-22 years, respectively; P = .017).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Mucinous cell proliferation associated with type 1 CPAM has exceptionally good long-term outcomes if confined within the same lobe of CPAM. A second oncogenic mutation in the GNAS gene may be necessary for progression to malignancy and distant spread.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ajcp\/aqaa245","type":"journal-article","created":{"date-parts":[[2020,10,28]],"date-time":"2020-10-28T16:12:08Z","timestamp":1603901528000},"page":"313-319","source":"Crossref","is-referenced-by-count":18,"title":["Mucinous Adenocarcinoma With Intrapulmonary Metastasis Harboring\n                    <i>KRAS<\/i>\n                    and\n                    <i>GNAS<\/i>\n                    Mutations Arising in Congenital Pulmonary Airway Malformation"],"prefix":"10.1093","volume":"156","author":[{"given":"Ximena Fernandez","family":"de Cordova","sequence":"first","affiliation":[{"name":"Universidad Aut\u00f3noma de Guadalajara School of Medicine, Guadalajara, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huiying","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Microbiology and Immunology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitra","family":"Mehrad","sequence":"additional","affiliation":[{"name":"Department of Pathology, Microbiology and Immunology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosana","family":"Eisenberg","sequence":"additional","affiliation":[{"name":"Department of Pathology, Microbiology and Immunology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joyce","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Pathology, Microbiology and Immunology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiang","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology\/Oncology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott","family":"Borinstein","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Vanderbilt University Medical Center, Nashville, TN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa E","family":"Danko","sequence":"additional","affiliation":[{"name":"Department of Molecular Physiology and Biophysics, Vanderbilt University, Nashville, TN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7215-4346","authenticated-orcid":false,"given":"Jiancong","family":"Liang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Microbiology and Immunology"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,2,20]]},"reference":[{"key":"2021070713343348000_CIT0001","volume-title":"Stocker and Dehner\u2019s Pediatric Pathology","author":"Husain","year":"2016"},{"key":"2021070713343348000_CIT0002","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1016\/S2352-4642(18)30035-X","article-title":"Congenital pulmonary airway malformation: advances and controversies","volume":"2","author":"Wong","year":"2018","journal-title":"Lancet Child Adolesc Health."},{"key":"2021070713343348000_CIT0003","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1053\/j.sempedsurg.2015.02.002","article-title":"The pathology of congenital lung lesions","volume":"24","author":"Fowler","year":"2015","journal-title":"Semin Pediatr Surg."},{"key":"2021070713343348000_CIT0004","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/S1055-8586(03)70004-3","article-title":"New concepts in the pathology of congenital lung malformations","volume":"12","author":"Langston","year":"2003","journal-title":"Semin Pediatr Surg."},{"key":"2021070713343348000_CIT0005","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/S0046-8177(77)80078-6","article-title":"Congenital cystic adenomatoid malformation of the lung. 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