title: Prognostic value of hedgehog signal component expressions in hepatoblastoma patients.
abstract: OBJECTIVE
  Activation of hedgehog (Hh) pathway has been implicated in the development of human malignancies. Hh as well as related downstream target genes has been extensively studied in many kinds of malignant tumours for clinical diagnostic or prognostic utilities. This study aimed at investigating whether Hh molecules provides a molecular marker of hepatoblastoma malignancy.
  
  
  METHODS
  We obtained tissue sections from 32 patients with hepatoblastoma as well as cholestasis and normal control. Immunohistochemical analysis were performed to determine Hh signal components in human hepatoblastoma. The prognostic significance of single expression of Hh signal components were evaluated using Cox proportional hazards regression models and Kaplan-Meier survival analysis for statistical analysis.
  
  
  RESULTS
  Expression of Hh signal components showed an increase in hepatoblastoma compared with cholestasis and normal tissues. There was a positive correlation between Smo or Gli1 expression and tumor clinicopathological features, such as histological type, tumor grade, tumor size and clinical stage. Both Smo or Gli1 protein high expression was significantly associated with poor prognosis by univariate analyses and multivariate analyses.
  
  
  CONCLUSIONS
  Abnormal Hh signaling activation plays important roles in the malignant potential of hepatoblastoma. Gli1 expression is an independent prognostic marker.
authors.affiliation: Department of Hepatobiliary Surgery, Children's Hospital of Chongqing Medical University, 22 Zhongshan Rd., Chongqing, 400014, PR China.
authors.forename: Ying-Cun
authors.initials: YC
authors.lastname: Li
authors.forename: Yu-Hua
authors.initials: YH
authors.lastname: Deng
authors.forename: Zhen-Hua
authors.initials: ZH
authors.lastname: Guo
authors.forename: Ming-Man
authors.initials: MM
authors.lastname: Zhang
authors.forename: Jin
authors.initials: J
authors.lastname: Zhu
authors.forename: Chon-Lun
authors.initials: CL
authors.lastname: Pu
authors.forename: Chun-Ping
authors.initials: CP
authors.lastname: Xiang
authors.forename: Chun-Bao
authors.initials: CB
authors.lastname: Guo
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D053823:Hedgehog Proteins / Q000502:physiology*; D018197:Hepatoblastoma / Q000401:mortality* / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000401:mortality* / Q000473:pathology; D008297:Male; D015999:Multivariate Analysis; D009367:Neoplasm Staging; D011379:Prognosis; D015398:Signal Transduction / Q000502:physiology*; D014157:Transcription Factors / Q000032:analysis*; D000071676:Zinc Finger Protein GLI1
chemical_list: C400551:GLI1 protein, human; D053823:Hedgehog Proteins; D014157:Transcription Factors; D000071676:Zinc Finger Protein GLI1