Malignant rhabdoid tumors (MRTs) of the central nervous system (atypical teratoid, rhabdoid tumor (AT/RT)), kidney (rhabdoid tumor of the kidney (RTK)), and soft tissues all share an aggressive clinical behavior and dismal prognosis. The burden of the intensive treatment required to cure patients is a matter of great concern given the young median age at diagnosis, regardless of tumor location. Thus, a greater understanding of the oncogenic properties of SMARCB1 and the SWI/SNF complex, as well as the clinical aspects of malignant rhabdoid tumors is necessary. Towards this aim, the first international SMARCB1 and rhabdoid tumor symposium was held in Paris, France in December 2013, organized by the collaborative efforts of the Dana-Farber Cancer Institute (Susan Chi), Swabian Children's Cancer Center/EU-RHAB Center (Michael Frühwald) and Curie Institute (Franck Bourdeaut). This workshop of physicians and other scientists fostered the integration of biologic insights towards clinical application in rhabdoid tumors, and other SWI/SNF-related cancers. The following report is a synopsis of the highlights of this meeting.
Keywords: ATRT; INI1; Rhabdoid; SMARCB1; SWI/SNF.
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