Atypical teratoid rhabdoid tumor diagnostic criteria
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
The diagnosis of atypical teratoid rhabdoid tumor is based on the current WHO classification of tumors of the central nervous system criteria, which include presence of rhabdoid tumor cells and/or divergent differentiation along epithelial, mesenchymal, neuronal, or glial lines in addition to the complete loss of SMARCB1 protein expression in tumor cell nuclei, but the expression retained in preexisting cells (e.g., endothelial cells).[1]
Diagnostic Criteria
The diagnosis of atypical teratoid rhabdoid tumor is based on the current WHO classification of tumors of the central nervous system criteria, which include:[1]
- Presence of rhabdoid tumor cells and/or divergent differentiation along epithelial, mesenchymal, neuronal, or glial lines
- AND
- Complete loss of SMARCB1 protein expression in tumor cell nuclei, but the expression retained in preexisting cells (e.g., endothelial cells)
References
- ↑ 1.0 1.1 Slavc, Irene; Chocholous, Monika; Leiss, Ulrike; Haberler, Christine; Peyrl, Andreas; Azizi, Amedeo A.; Dieckmann, Karin; Woehrer, Adelheid; Peters, Christina; Widhalm, Georg; Dorfer, Christian; Czech, Thomas (2014). "Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012". Cancer Medicine. 3 (1): 91–100. doi:10.1002/cam4.161. ISSN 2045-7634.