title: Analysis of the neoplastic nature and biological potential of sporadic and nevoid basal cell carcinoma syndrome-associated keratocystic odontogenic tumor.
abstract: BACKGROUND
  Keratocystic odontogenic tumor (KCOT), also known as odontogenic keratocyst, is a benign cystic neoplasm, which may be associated with nevoid basal cell carcinoma syndrome (NBCCS) and if it does, will occur as multiple cystic lesions. KCOT is locally destructive despite its bland histological features. However, the neoplastic nature of KCOT is not well established. Heparanase is an endo-d-glucuronidase enzyme that specifically cleaves heparan sulfate (HS) and the increase of its level in tumors promotes invasion, angiogenesis, and metastasis.
  
  
  METHODS
  To investigate the neoplastic character of KCOT, we studied the localization patterns of heparanase in KCOT, focusing on the differences between sporadic and NBCCS-associated KCOTs, by immunohistochemistry and in situ hybridization. To compare the expression pattern of these cysts with non-tumorous odontogenic developmental cyst, dentigerous cyst was included.
  
  
  RESULTS
  All the odontogenic cysts showed positive immunoreaction for heparanase protein in various intensities. The expression pattern of heparanase gene corresponded to that of protein expression. Interestingly, intense gene and protein expressions were observed in KCOT associated with NBCCS compared with sporadic ones and dentigerous cyst.
  
  
  CONCLUSIONS
  The results implied that heparanase expression may be correlated with the neoplastic properties of KCOT, particularly in NBCCS-associated cases.
authors.affiliation: Department of Oral Pathology and Medicine, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Shikata-cho, Okayama, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Katase
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Nagatsuka
authors.forename: Hidetsugu
authors.initials: H
authors.lastname: Tsujigiwa
authors.forename: Mehmet
authors.initials: M
authors.lastname: Gunduz
authors.forename: Ryo
authors.initials: R
authors.lastname: Tamamura
authors.forename: Han Phuu
authors.initials: HP
authors.lastname: Pwint
authors.forename: Rosario Santos
authors.initials: RS
authors.lastname: Rivera
authors.forename: Motowo
authors.initials: M
authors.lastname: Nakajima
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Naomoto
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Nagai
mesh_terms: D001478:Basal Cell Nevus Syndrome / Q000150:complications / Q000201:enzymology*; D003803:Dentigerous Cyst / Q000201:enzymology; D015972:Gene Expression Regulation, Neoplastic; D005966:Glucuronidase / Q000096:biosynthesis* / Q000235:genetics; D019812:Heparan Sulfate Proteoglycans / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D009361:Neoplasm Invasiveness; D009807:Odontogenic Cysts / Q000150:complications / Q000201:enzymology*; D009808:Odontogenic Tumors / Q000150:complications / Q000201:enzymology*; D000099175:Heparanase
chemical_list: D005966:Glucuronidase; D019812:Heparan Sulfate Proteoglycans; D000099175:Heparanase