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000287018 1001_ $$aHasselblatt, Martin$$b0
000287018 245__ $$aSWI/SNF-deficient tumors of the central nervous system: An update.
000287018 260__ $$aDeisenhofen, München$$bDustri-Verl.$$c2024
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000287018 500__ $$a2024 Jan-Feb;43(1):2-9
000287018 520__ $$aAtypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant tumor of the central nervous system characterized by biallelic inactivation of SWI/SNF chromatin remodeling complex members SMARCB1/INI1 or (rarely) SMARCA4/BRG1. Most high-grade central nervous system lesions showing loss of nuclear SMARCB1 or SMARCA4 protein expression can indeed be categorized as AT/RT. However, some high-grade lesions have been identified, whose clinical and/or molecular features justify separation from AT/RT. Furthermore, other recently described tumor types such as desmoplastic myxoid tumor, SMARCB1-mutant, and low-grade diffusely infiltrative tumor, SMARCB1-mutant, may even manifest as low-grade lesions. Here, we review recent developments in the definition of the molecular landscape of AT/RT and give an update on other rare high- and low-grade SWI/SNF-deficient central nervous system tumors.
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000287018 7001_ $$aFrühwald, Michael C$$b2
000287018 773__ $$0PERI:(DE-600)2007408-6$$a10.5414/NP301594$$n1$$p2-9$$tClinical neuropathology$$v43$$x0722-5091$$y2024
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