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]

Diagnostic Criteria

The diagnosis of atypical teratoid rhabdoid tumor is based on the current WHO classification of tumors of the CNS criteria, which include:

• 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 expression retained in preexisting cells (e.g., endothelial cells)