{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T14:35:23Z","timestamp":1789396523875,"version":"build-2803163510"},"reference-count":32,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2006,5,1]],"date-time":"2006-05-01T00:00:00Z","timestamp":1146441600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2006,5]]},"abstract":"<jats:p>Congenital cystic adenomatoid malformation (CCAM), a developmental anomaly of lung, shares many features with the pediatric tumor pleuropulmonary blastoma (PPB). Both may show benign epithelium-lined cysts and mesenchymal proliferation, often with skeletal muscle differentiation. Before its recognition as a distinct entity, PPB was described in several reports as \u201crhabdomyosarcoma arising in CCAM.\u201d Abnormal karyotypes in PPB often show excess material from chromosome 8. It has also been suggested that PPB may harbor p53 mutations. We examined the karyotype and searched for p53 mutations (via immunostaining and single-strand conformation polymorphism analysis) in 11 CCAM and in 2 PPB. Karyotypes were normal in all CCAM and showed clonal abnormalities in both PPB. There was marked and diffuse immunopositivity for nuclear p53 in the epithelial cells of CCAM and PPB. Strong staining was also observed in approximately 50% of the stromal cells in all PPB, but was seen in the stroma of only 2 of 10 CCAM, where it was faint and focal. TP53 mutations were not identified in CCAM or PPB. We conclude that CCAM does not contain the clonal chromosomal aberrations reported in PPB and shows less stromal p53 immunostaining than PPB. Since p53 mutations were not identified in either entity, the observed p53 immunoreactivity may be caused by another mechanism; its role in PPB and CCAM pathogenesis remains to be determined. Overall, these findings provide evidence that CCAM is nonneoplastic. Although some may view CCAM as a PPB precursor, it remains biologically distinct in terms of karyotype and p53 status.<\/jats:p>","DOI":"10.2350\/06-01-0025.1","type":"journal-article","created":{"date-parts":[[2006,7,12]],"date-time":"2006-07-12T17:06:26Z","timestamp":1152723986000},"page":"190-195","source":"Crossref","is-referenced-by-count":29,"title":["Cytogenetic and p53 Profiles in Congenital Cystic Adenomatoid Malformation: Insights into its Relationship with Pleuropulmonary Blastoma"],"prefix":"10.1177","volume":"9","author":[{"given":"Sara O.","family":"Vargas","sequence":"first","affiliation":[{"name":"Department of Pathology, Children's Hospital Boston, Boston, MA, USA"},{"name":"Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA"},{"name":"Department of Pathology, Harvard Medical School, Boston, MA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esther","family":"Korpershoek","sequence":"additional","affiliation":[{"name":"Department of Pathology, Josephine Nefkens Institute, Erasmus Medical Center, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harry P.W.","family":"Kozakewich","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children's Hospital Boston, Boston, MA, USA"},{"name":"Department of Pathology, Harvard Medical School, Boston, MA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald R.","family":"de Krijger","sequence":"additional","affiliation":[{"name":"Department of Pathology, Josephine Nefkens Institute, Erasmus Medical Center, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan A.","family":"Fletcher","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children's Hospital Boston, Boston, MA, USA"},{"name":"Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA"},{"name":"Department of Pathology, Harvard Medical School, Boston, MA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio R.","family":"Perez-Atayde","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children's Hospital Boston, Boston, MA, USA"},{"name":"Department of Pathology, Harvard Medical School, Boston, MA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2006,5,1]]},"reference":[{"key":"bibr1-06-01-0025.1","doi-asserted-by":"publisher","DOI":"10.1007\/s10024-003-4044-z"},{"key":"bibr2-06-01-0025.1","doi-asserted-by":"publisher","DOI":"10.1016\/S1055-8586(03)70004-3"},{"key":"bibr3-06-01-0025.1","doi-asserted-by":"publisher","DOI":"10.5858\/2002-126-0934-CACICT"},{"key":"bibr4-06-01-0025.1","unstructured":"Riedlinger WFJ, Vargas SO, Jennings RW, Bronchial atresia is common to extralobar sequestration, intralobar sequestration, cystic adenomatoid malformation and lobar emphysema. 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