title: The expression of vascular endothelial growth factor and its receptors in congenital bronchopulmonary cystic malformations.
abstract: BACKGROUND
  Bronchopulmonary sequestration (BPS) and congenital cystic adenomatoid malformation (CCAM) represent rare hamartomatous abnormalities of the lung. Dysregulation of cytokines that influence pulmonary vasculogenesis and epithelial growth, both known to be altered in BPS and CCAM, may play a role in their pathogenesis.
  
  
  OBJECTIVE
  We hypothesized that expression of vascular endothelial growth factor (VEGF) or its receptors might be altered in CCAM and BPS, possibly distinguishing CCAM from BPS, or from controls.
  
  
  METHODS
  Lung biopsy specimens obtained from infants who had undergone surgery for BPS (n = 4) or CCAM (n = 5) within the first month of life and normal lung autopsy samples (n = 4) serving as controls were investigated immunohistochemically for the protein expression levels of VEGF and its corresponding receptors.
  
  
  RESULTS
  VEGF, vascular endothelial growth factor receptor 1 (VEGFR1), vascular endothelial growth factor receptor 2 (VEGFR2), and vascular endothelial growth factor receptor 3 (VEGFR3) staining was detected in CCAM and BPS specimens, as well as in control samples. VEGFR2 expression increased from controls to CCAM and from CCAM to BPS, the difference between controls and BPS being significant. The expression of VEGF, VEGFR1, and VEGFR3 was similar among the three groups. Consistent with a possible involvement of VEGFR2 in altered vasculogenesis-bronchiogenesis interaction, its expression was predominantly found in bronchial but not alveolar regions.
  
  
  CONCLUSIONS
  The data suggest a possible role of VEGF-VEGFR2 interaction in the pathogenesis of congenital bronchopulmonary cystic malformations. However, VEGFR2 does not represent a suitable histochemical marker to distinguish between BPS and CCAM.
authors.affiliation: Department of Neonatology, Charité - Universitätsmedizin Berlin, Berlin, Germany. sven.weber@charite.de
authors.forename: Sven C
authors.initials: SC
authors.lastname: Weber
authors.forename: Hannes
authors.initials: H
authors.lastname: Sallmon
authors.forename: Nanette
authors.initials: N
authors.lastname: Sarioglu
authors.forename: Petra
authors.initials: P
authors.lastname: Degenhardt
authors.forename: Christoph
authors.initials: C
authors.lastname: Bührer
authors.forename: Mario
authors.initials: M
authors.lastname: Rüdiger
authors.forename: Petra
authors.initials: P
authors.lastname: Koehne
mesh_terms: D001706:Biopsy; D001998:Bronchopulmonary Sequestration / Q000378:metabolism*; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000378:metabolism*; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000473:pathology; D008297:Male; D040262:Receptors, Vascular Endothelial Growth Factor / Q000378:metabolism*; D042461:Vascular Endothelial Growth Factor A / Q000378:metabolism*
chemical_list: D042461:Vascular Endothelial Growth Factor A; D040262:Receptors, Vascular Endothelial Growth Factor