J Korean Neurosurg Soc.  2002 Dec;32(6):599-602.

Atypical Teratoid/Rhabdoid Tumor in Central Nervous System: Report of 2 Cases

Affiliations
  • 1Department of Neurological Surgery, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea. ysra@amc.seoul.kr
  • 2Department of Pediatrics, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.
  • 3Department of Pathology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.

Abstract

We report two cases of atypical teratoid/rhabdoid tumor(AT/RT) in the central nervous system. Primary central nervous system AT/RT is an extremely rare malignant tumor affecting infants and young children. These tumors have been diagnosed previously as primitive neuroectodermal tumors(PNETs) because AT/RT contains fields indistinguishable from classic PNETs. Separation of these two tumor types is crucial because the prognosis for AT/RT is poor even when treatment includes surgery with or without radiation therapy and/or chemotherapy. Clinical, radiological and histopathological features of AT/RT are presented.

Keyword

Atypical teratoid/rhabdoid tumor; Surgery; Child; Chemotherapy

MeSH Terms

Central Nervous System*
Child
Drug Therapy
Humans
Infant
Neural Plate
Neuroectodermal Tumors, Primitive
Prognosis
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