title: Heparanase-1 and components of the hedgehog signalling pathway are increased in untreated alveolar orbital rhabdomyosarcoma.
abstract: BACKGROUND
  To assess the activities of heparanase-1 and elements of the hedgehog signalling pathway in alveolar orbital rhabdomyosarcoma.
  
  
  METHODS
  Specimens (n = 23) were divided into two groups, those from patients with preoperative chemoradiotherapy or untreated patients; six samples of normal extraocular muscle were used as a normal muscle group. The presence of heparanase-1, patched, smoothened and glioma-associated oncogene homologue-1 protein expression was determined in 23 cases of archival paraffin-embedded alveolar orbital rhabdomyosarcoma after immunohistochemistry. RNA was extracted from three groups of paraffin-embedded specimens and messenger RNA expressions of heparanase-1, smoothened and glioma-associated oncogene homologue-1 compared using nested reverse transcriptase polymerase chain reaction and a limiting dilution analysis.
  
  
  RESULTS
  The heparanase-1, patched, smoothened and glioma-associated oncogene homologue-1 protein was expressed in 91.3%, 87.0%, 91.3% and 78.3%, respectively, of the alveolar orbital rhabdomyosarcoma specimens. Untreated rhabdomyosarcoma samples tended to stain intensely, but staining was relatively weak in tissue obtained from the chemoradiotherapy group. The expression levels of heparanase-1, smoothened and glioma-associated oncogene homologue-1 messenger RNA in untreated and chemoradiotherapy groups paralleled that seen with immunology, and there were no significant differences in heparanase-1, smoothened and glioma-associated oncogene homologue-1 messenger RNA levels between the chemoradiotherapy group and the normal muscle group (P > 0.05). However, the messenger RNA in the untreated group were all significantly higher than those in the chemoradiotherapy and normal muscle groups (P < 0.01).
  
  
  CONCLUSIONS
  Both heparanase-1 and hedgehog signalling pathway are involved in the pathogenesis of alveolar orbital rhabdomyosarcoma; however, chemotherapy and/or radiotherapy appears to significantly inhibit their upregulation.
authors.affiliation: Department of Ophthalmology, the First Affiliated Hospital of General Hospital of People's Liberation Army, Beijing, China.
authors.forename: Wei-Qiang
authors.initials: WQ
authors.lastname: Tang
authors.forename: Yan
authors.initials: Y
authors.lastname: Hei
authors.forename: Li
authors.initials: L
authors.lastname: Kang
authors.forename: Li-Hua
authors.initials: LH
authors.lastname: Xiao
mesh_terms: D000293:Adolescent; D000328:Adult; D000818:Animals; D059248:Chemoradiotherapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005966:Glucuronidase / Q000235:genetics / Q000378:metabolism*; D053823:Hedgehog Proteins / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D009918:Orbital Neoplasms / Q000201:enzymology* / Q000628:therapy; D000072079:Patched Receptors; D012333:RNA, Messenger / Q000378:metabolism; D011956:Receptors, Cell Surface / Q000235:genetics / Q000378:metabolism; D043562:Receptors, G-Protein-Coupled / Q000235:genetics / Q000378:metabolism; D020133:Reverse Transcriptase Polymerase Chain Reaction; D018232:Rhabdomyosarcoma, Alveolar / Q000201:enzymology* / Q000628:therapy; D015398:Signal Transduction; D000072086:Smoothened Receptor; D014157:Transcription Factors / Q000235:genetics / Q000378:metabolism; D000071676:Zinc Finger Protein GLI1; D000099175:Heparanase
chemical_list: D005966:Glucuronidase; D053823:Hedgehog Proteins; D000072079:Patched Receptors; D012333:RNA, Messenger; D011956:Receptors, Cell Surface; D043562:Receptors, G-Protein-Coupled; D000072086:Smoothened Receptor; D014157:Transcription Factors; D000071676:Zinc Finger Protein GLI1; D000099175:Heparanase; C400551:GLI1 protein, human; C491023:SMO protein, human
keywords: hedgehog; heparanase-1; orbital rhabdomyosarcoma.