Atypical teratoid/rhabdoid tumors of central nervous system

Aims and Objectives: To review the clinical features and current understanding of the epidemiology, biology and management of pediatric atypical teratoid/rhabdoid tumors and analyzing the different treatment modalities. Materials and Methods: The MEDLINE database, bibliographies of selected articles and current English-language texts on the subject were reviewed. A Pubmed search was made with keywords pediatric atypical teratoid/rhabdoid tumors, intracranial, surgery, chemotherapy and radiotherapy. Most recent articles and also significant older articles having all above said words were selected and their results were reviewed in detail. Results: Atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system (CNS) most frequently diagnosed in smaller than 3 years of age and incidence is 1-2% of all brain tumors in children. 63% of the AT/RT of the CNS is seen in infra-tentorial compartment, there are no precise imaging features that differentiate AT/RT from the other posterior fossa tumor. The "rhabdoid" cells are characteristic on cytopathology. It has been established now that CNS, AT/RT often show deletion of the long arm of chromosome 22q11.2. The initial treatment for most children with AT/RT is surgical with and without cerebrospinal fluid diversionary procedure. Children with less than 3 years of age offered chemotherapy but in older children radiotherapy is given in addition.