INI-1 SMARCB1

Identifies the gene product of the INI-1 gene, a tumor suppressor gene, and inactivation through mutation or other mechanism characterizes a unique subset of tumors that includes renal and extrarenal rhabdoid tumors, atypical teratoid/rhabdoid tumors of the central nervous system, distal and proximal variants of epithelioid sarcoma, as well as a subset of mesenchymal chondrosarcomas, epithelioid malignant peripheral nerve sheath tumors, and soft tissue myoepitheliomas. A lack of nuclear expression of INI1 is characteristic of malignant rhabdoid tumors and epithelioid sarcomas.