Atypical Teratoid Rhabdoid Tumors

  • Lucie Lafay-Cousin
  • Douglas R. Strother
  • Jennifer A. Chan
  • Jonathon Torchia
  • Annie Huang
Chapter

Abstract

Atypical teratoid rhabdoid tumor of the brain is a rare embryonal tumor of infancy which has become more widely recognized due to specific immunostaining now more routinely available. These tumors still carry a poor prognosis and no standard therapy is currently available. However, the recent development of aggressive multimodality strategies seems to be associated with improved survival rates. In this chapter, we provide a review of our current knowledge on the demographics and epidemiology, pathology, and molecular characteristics. We review each individual therapeutic modality used (surgery, conventional chemotherapy, high-dose chemotherapy with stem cell rescue, intrathecal chemotherapy, and radiation) and the contribution of each to outcome when available. We provide an overview of the most recent therapeutic trials published and discuss the future options in development.

Keywords

Atypical teratoid rhabdoid tumor Embryonal tumor Infancy INI1 High-dose chemotherapy Radiation 
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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  • Lucie Lafay-Cousin
    • 1
  • Douglas R. Strother
    • 2
  • Jennifer A. Chan
    • 3
  • Jonathon Torchia
    • 4
  • Annie Huang
    • 5
  1. 1.Department of Pediatrics, Division of OncologyAlberta Children’s HospitalCalgaryCanada
  2. 2.Departments of Oncology and Pediatrics, Section of Oncology and Blood and Marrow TransplantCumming School of Medicine and Alberta Children’s HospitalCalgaryCanada
  3. 3.Department of Pathology & Laboratory MedicineUniversity of CalgaryCalgaryCanada
  4. 4.Department of Lab Medicine and Pathobiology, Labatt’s Brain Tumour Centre, SickKidsUniversity of TorontoTorontoCanada
  5. 5.Department of Cell BiologyThe Hospital for Sick ChildrenTorontoCanada

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