rhabdoid tumour predisposition syndrome type 1

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rhabdoid tumour predisposition syndrome type 1

An inherited predisposition (OMIM:609322) to develop rhabdoid tumours, a highly malignant group of neoplasms that usually occur in early childhood. 

Molecular pathology
Defects in SMARCB1, which encodes a core component of the ATP-dependent chromatin-remodelling BAF (hSWI/SNF) complex with key roles in cell proliferation and differentiation, cause rhabdoid tumour predisposition syndrome type 1.