title: Expression of HIF-1 regulated proteins vascular endothelial growth factor, carbonic anhydrase IX and hypoxia inducible gene 2 in hemangioblastomas.
abstract: BACKGROUND
  Hemangioblastomas occur as sporadic or as von Hippel-Lindau syndrome (VHL) associated tumors. In both settings, activation of the VHL-HIF-1 (hypoxia induced factor) pathway is thought to be important in tumor biology.
  
  
  METHODS
  We performed immunohistochemical studies on 23 hemangioblastomas, 13 meningiomas and 4 hemangiopericytomas to evaluate expression of the VHL-HIF-1 regulated proteins vascular endothelial growth factor (VEGF), carbonic anhydrase IX (CAIX) and hypoxia inducible gene 2 (HIG-2).
  
  
  RESULTS
  Hemangioblastomas showed significantly higher expression of CAIX and VEGF than the other tested tumors. They showed strong membranous expression of CAIX in a pattern identical to that observed in clear cell renal cell carcinomas (CCRCC). Interestingly, hemangioblastomas lacked significant reactivity for HIG-2.
  
  
  CONCLUSIONS
  The VHL-HIF-1 regulated genes VEGF and CAIX are expressed in hemangioblastomas but significant HIG-2 expression is not observed. Carbonic anhydrase IX staining in particular may be a helpful marker of hemangioblastomas but does not aid in the distinction from CCRCC.
authors.affiliation: Peter Pytel, MD, Department of Pathology, MC6101, Room E609, 5841 S. Maryland Ave, Chicago, IL 60637, USA, phone: (773) 795-6751, fax: (773) 702-1243, e-mail: peter.pytel@uchospitals.edu.
authors.forename: Mei
authors.initials: M
authors.lastname: Li
authors.forename: Jie
authors.initials: J
authors.lastname: Song
authors.forename: Peter
authors.initials: P
authors.lastname: Pytel
mesh_terms: D000951:Antigens, Neoplasm / Q000032:analysis / Q000096:biosynthesis*; D014408:Biomarkers, Tumor / Q000032:analysis; D000071231:Carbonic Anhydrase IX; D002256:Carbonic Anhydrases / Q000032:analysis / Q000096:biosynthesis*; D002528:Cerebellar Neoplasms / Q000378:metabolism*; D018325:Hemangioblastoma / Q000378:metabolism*; D006801:Humans; D051793:Hypoxia-Inducible Factor 1 / Q000235:genetics / Q000378:metabolism*; D007150:Immunohistochemistry; D009363:Neoplasm Proteins / Q000032:analysis / Q000096:biosynthesis*; D042461:Vascular Endothelial Growth Factor A / Q000032:analysis / Q000096:biosynthesis*
chemical_list: D000951:Antigens, Neoplasm; D014408:Biomarkers, Tumor; C405620:HILPDA protein, human; D051793:Hypoxia-Inducible Factor 1; D009363:Neoplasm Proteins; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; C089226:CA9 protein, human; D000071231:Carbonic Anhydrase IX; D002256:Carbonic Anhydrases