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Atypical teratoid/rhabdoid tumors-current concepts, advances in biology, and potential future therapies

Michael C Frühwald1, Jaclyn A Biegel1, Franck Bourdeaut1

  • 1Children's Hospital and Swabian Children's Cancer Center, Augsburg, Germany (M.C.F.); Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, California (J.A.B.); INSERM U830, Laboratory of Genetics and Biology of Cancers, and Department of Pediatric Oncology, Curie Institute, Paris, France (F.B.); Comprehensive Cancer Center and Department of Oncology, St Jude Children's Research Hospital, Memphis, Tennessee (C.W.M.R.); Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts (S.N.C.); Division of Hematology/Oncology, Boston Children's Hospital, Boston, Massachusetts (S.N.C.); Department of Pediatrics, Harvard Medical School, Boston, Massachusetts (S.N.C.).

Neuro-Oncology
|January 13, 2016
PubMed

Insights

Atypical teratoid/rhabdoid tumors (AT/RT) in young children often involve epigenetic regulators like SMARCB1. Understanding these epigenetic changes may reveal new therapeutic targets beyond conventional chemotherapy.

Area of Science:

  • Pediatric oncology
  • Molecular biology
  • Epigenetics

Background:

  • Atypical teratoid/rhabdoid tumor (AT/RT) is a rare and aggressive brain tumor predominantly affecting infants.
  • Genomic alterations in the SWItch/sucrose nonfermentable (SWI/SNF) chromatin remodeling complex, particularly SMARCB1, are hallmarks of AT/RT.
  • Emerging evidence suggests molecular heterogeneity within AT/RT, indicating distinct subgroups.

Purpose of the Study:

  • To explore the role of epigenetic dysregulation in the pathogenesis of AT/RT.
  • To identify potential molecular targets for novel therapeutic strategies in AT/RT.
  • To understand the basis for chemotherapy resistance in a subset of AT/RT patients.

Main Methods:

  • Genomic profiling (transcription and methylation) to identify molecular subgroups.
  • Analysis of epigenetic regulatory networks in AT/RT.
  • Review of signaling pathways implicated in AT/RT pathogenesis.

Main Results:

  • AT/RT pathogenesis is linked to a complex network of epigenetic factors.
  • Specific molecular subgroups within AT/RT may exist.
  • Several aberrant signaling pathways (e.g., Wnt, Hedgehog) are identified as potential therapeutic targets.

Conclusions:

  • Epigenetic dysregulation is central to AT/RT development.
  • Targeting specific molecular pathways offers a promising avenue for novel AT/RT therapies.
  • Further research into epigenetic mechanisms is crucial for improving treatment outcomes in AT/RT.
Keywords:
AT/RTBAF47INI1SMARCB1SNF5SWI/SNFepigeneticsrhabdoid tumor

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