Journal of Neuro-Oncology

, Volume 72, Issue 1, pp 77–84

Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor

Authors

  • Mary Ann Zimmerman
    • Department of Pediatric Oncology, Pediatric Neuro-OncologyDana-Farber Cancer Institute
    • Division of Hematology/OncologyChildren’s Hospital
  • Liliana C. Goumnerova
    • Department of NeurosurgeryChildren’s Hospital
  • Mark Proctor
    • Department of NeurosurgeryChildren’s Hospital
  • R. Michael Scott
    • Department of NeurosurgeryChildren’s Hospital
  • Karen Marcus
    • Department of Radiation OncologyChildren’s Hospital
  • Scott L. Pomeroy
    • Department of NeurologyChildren’s Hospital
  • Christopher D. Turner
    • Department of Pediatric Oncology, Pediatric Neuro-OncologyDana-Farber Cancer Institute
    • Division of Hematology/OncologyChildren’s Hospital
  • Susan N. Chi
    • Department of Pediatric Oncology, Pediatric Neuro-OncologyDana-Farber Cancer Institute
    • Division of Hematology/OncologyChildren’s Hospital
  • Christine Chordas
    • Department of Pediatric Oncology, Pediatric Neuro-OncologyDana-Farber Cancer Institute
    • Division of Hematology/OncologyChildren’s Hospital
    • Department of Pediatric Oncology, Pediatric Neuro-OncologyDana-Farber Cancer Institute
    • Division of Hematology/OncologyChildren’s Hospital
Clinical Study

DOI: 10.1007/s11060-004-3115-y

Cite this article as:
Ann Zimmerman, M., Goumnerova, L.C., Proctor, M. et al. J Neurooncol (2005) 72: 77. doi:10.1007/s11060-004-3115-y

Abstract

Atypical teratoid/rhabdoid tumors (AT/RT) are highly malignant lesions of childhood that carry a very poor prognosis. AT/RT can occur in the central nervous system (CNS AT/RT) and disease in this location carries an even worse prognosis with a median survival of 7 months. In spite of multiple treatment regimens consisting of maximal surgical resection (including second look surgery), radiation therapy (focal and craniospinal), and multi-agent intravenous, oral and intrathecal chemotherapy, with or without high-dose therapy and stem cell rescue, only seven long-term survivors of CNS AT/RT have been reported, all in patients with newly diagnosed disease. For this reason, many centers now direct such patients, particularly those under 5 years of age, or those with recurrent disease, towards comfort care rather than attempt curative therapy. We now report on four children, two with newly diagnosed CNS AT/RT and two with progressive disease after multi-agent chemotherapy who are long term survivors (median follow-up of 37 months) using a combination of surgery, radiation therapy, and intensive chemotherapy. The chemotherapy component was modified from the Intergroup Rhabdomyosarcoma Study Group (IRS III) parameningeal protocol as three of the seven reported survivors in the literature were treated using this type of therapy. Our four patients, when added to the three reported survivors in the literature using this approach, suggest that patients provided this aggressive therapy can significantly alter the course of their disease. More importantly, we report on the first two survivors after relapse with multi-agent intravenous and intrathecal chemotherapy treated with this modified regimen.

Keywords

atypical teratoid/rhabdoid tumor (AT/RT) brain tumor pediatric rhabdoid treatment

Copyright information

© Springer 2005