Hedgehog pathway activity in pediatric embryonal rhabdomyosarcoma and undifferentiated sarcoma: a report from the Children's Oncology Group

Joseph G Pressey, James R Anderson, David K Crossman, James C Lynch, Frederic G Barr

Research output: Contribution to journalArticle

51 Citations (Scopus)

Abstract

Aberrant activation of the hedgehog (Hh) signaling pathway is implicated widely in both pediatric and adult malignancies. Inactivation of the Hh regulator PTCH is responsible for the Gorlin cancer predisposition syndrome. The spectrum of tumors found in Gorlin Syndrome includes basal cell carcinoma, medulloblastoma, and rarely, rhabdomyosarcoma (RMS). A previous report utilizing in situ hybridization has provided initial evidence for the expression of Hh targets GLI1 and PTCH in RMS tumors.
Original languageEnglish
Pages (from-to)930-8
Number of pages9
JournalPediatric Blood & Cancer
Volume57
Issue number6
DOIs
Publication statusPublished - 1 Dec 2011

Keywords

  • Cell Line, Tumor
  • Hedgehog Proteins
  • Humans
  • Oligonucleotide Array Sequence Analysis
  • RNA, Messenger
  • Receptors, Cell Surface
  • Rhabdomyosarcoma
  • Sarcoma
  • Signal Transduction
  • Transcription Factors

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