Two Cases of Central Nervous System Atypical Teratoid/Rhabdoid Tumors / 대한소아혈액종양학회지
Korean Journal of Pediatric Hematology-Oncology
;
: 293-298, 2000.
Article
in Korean
| WPRIM
| ID: wpr-113919
ABSTRACT
Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) of infancy and childhood, a new entity among malignant pediatric brain tumors, is unique histologic entity with an extremely aggressive natural history. Histologically AT/RT is defined as a polymorphous neoplasm often featuring rhabdoid, primitive neuroectodermal tumor, epithelial, and mesenchymal components. AT/RT occurs mainly in the posterior fossa, so mimics medulloblastoma. AT/RT is characterized by the cytogenetic finding of monosomy 22 rather than i (17q). Standard chemotherapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective. Most children survive less than 12 months (mean survival 8.5 months) after diagnosis. We report two cases of central nervous system AT/RT in young children, one of them is alive so far with multimodal treatment.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Brain Neoplasms
/
Central Nervous System
/
Neuroectodermal Tumors, Primitive
/
Combined Modality Therapy
/
Natural History
/
Cytogenetics
/
Diagnosis
/
Drug Therapy
/
Medulloblastoma
/
Monosomy
Type of study:
Diagnostic study
Limits:
Child
/
Humans
/
Infant
Language:
Korean
Journal:
Korean Journal of Pediatric Hematology-Oncology
Year:
2000
Type:
Article
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