title: Differentiation of vascular tumors from vascular malformations by expression of Wilms tumor 1 gene: evaluation of 126 cases.
abstract: BACKGROUND
  Vascular tumors and malformations can be challenging to diagnose. Although they may initially appear very similar, they have distinct clinical courses and management. Wilms tumor 1 (WT1) gene expression has been reported in many different tumors including hematologic malignancies and some solid tumors.
  
  
  OBJECTIVE
  We sought to evaluate the expression of WT1 in 126 vascular lesions (64 vascular tumors, one Masson tumor, and 61 vascular malformations).
  
  
  METHODS
  Based on the International Society for the Study of Vascular Anomalies classification of vascular anomalies, we studied the expression of WT1 in vascular tumors composed of infantile hemangioma, congenital hemangiomas (non-involuting, rapidly involuting, and not otherwise specified), pyogenic granuloma, tufted angioma, cherry angioma, Kaposi sarcoma, and angiosarcoma. We also studied WT1 expression in vascular malformations composed of angiokeratoma/verrucous hemangioma, combined vascular malformations, venous malformations, glomuvenous malformations, lymphatic malformations/lymphangioma, telangiectasia, and targetoid hemosiderotic hemangioma.
  
  
  RESULTS
  All vascular tumors and proliferations had positive WT1 cytoplasmic endothelial immunostaining whereas only 3 vascular malformations were WT1 positive. Moreover the positivity of WT1 in these vascular malformations was focal and involved only re-endothelialized neovessels within thrombi.
  
  
  CONCLUSIONS
  The low number of malignant vascular tumors is a limitation.
  
  
  CONCLUSIONS
  Immunohistochemical detection of WT1 could be a useful tool to routine evaluation of vascular anomalies allowing the distinction of vascular tumors and proliferations from vascular malformations. Staining for WT1 may guide the clinician in difficult cases, as positive results would suggest a proliferative vascular lesion whereas negative results might point to a vascular malformation.
authors.affiliation: Division of Dermatology, Centre Hospitalier Universitaire Sainte-Justine, University of Montreal, Montreal, Quebec, Canada. roladhaybi@yahoo.com
authors.forename: Rola
authors.initials: R
authors.lastname: Al Dhaybi
authors.forename: Julie
authors.initials: J
authors.lastname: Powell
authors.forename: Catherine
authors.initials: C
authors.lastname: McCuaig
authors.forename: Victor
authors.initials: V
authors.lastname: Kokta
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001165:Arteriovenous Malformations / Q000378:metabolism / Q000473:pathology*; D014408:Biomarkers, Tumor / Q000378:metabolism*; D001706:Biopsy; D018797:Cell Cycle Proteins; D002455:Cell Division / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D003593:Cytoplasm / Q000378:metabolism; D003937:Diagnosis, Differential; D006391:Hemangioma / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D008875:Middle Aged; D009687:Nuclear Proteins / Q000378:metabolism*; D000072260:RNA Splicing Factors; D019043:Vascular Neoplasms / Q000378:metabolism / Q000473:pathology*; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D018797:Cell Cycle Proteins; D009687:Nuclear Proteins; D000072260:RNA Splicing Factors; C419554:WTAP protein, human