REVIEW ARTICLE
Atypical teratoid/rhabdoid tumor: short clinical description and insight into possible mechanism of the disease
B. Bikowska,
W. Grajkowska,
J. Jóźwiak,
B. Bikowska
Department of Histology and Embryology, Center for Biostructure Research, Medical University of Warsaw, Warsaw, Poland
Search for more papers by this authorW. Grajkowska
Department of Pathology, Children’s Memorial Health Institute, Warsaw, Poland
Search for more papers by this authorJ. Jóźwiak
Department of Histology and Embryology, Center for Biostructure Research, Medical University of Warsaw, Warsaw, Poland
Search for more papers by this authorB. Bikowska,
W. Grajkowska,
J. Jóźwiak,
B. Bikowska
Department of Histology and Embryology, Center for Biostructure Research, Medical University of Warsaw, Warsaw, Poland
Search for more papers by this authorW. Grajkowska
Department of Pathology, Children’s Memorial Health Institute, Warsaw, Poland
Search for more papers by this authorJ. Jóźwiak
Department of Histology and Embryology, Center for Biostructure Research, Medical University of Warsaw, Warsaw, Poland
Search for more papers by this authorJ. Jóźwiak, Department of Histology and Embryology, Center for Biostructure Research, Medical University of Warsaw, ul. Chalubinskiego 5, 02-004 Warsaw, Poland (tel.: +48 608 33 50 90; fax: +48 22 629 52 82; e-mail: [email protected]).
Abstract
Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant tumor typically appearing in childhood. Differentiation of AT/RT from other brain tumors is extremely important because of grim prognosis and necessity of more aggressive treatment. On the other hand, investigation is essential for new therapeutic agents based on continuously developing knowledge of AT/RT development mechanisms. Most AT/RT tumors have been demonstrated to harbor a chromosome 22 mutation in the region of hSNF5/INI1 gene, whose protein product participates in chromatin remodeling. Although the presence of this mutation is rather undisputable, additional molecular pathways underlying AT/RT development are poorly understood. Current paper discusses current views on molecular pathophysiology of the tumor.
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