Tumors with loss of the SMARCB1/INI1 protein include myoepithelial carcinomas, malignant rhabdoid tumors, atypical teratoid tumors of the central nervous system, renal medullary carcinomas, a subset of malignant peripheral nerve sheath tumors, and extraskeletal myxoid chondrosarcomas, while reduced nuclear expression has also been described in synovial sarcomas [9, 10]. This evidence concerns the gene SMARCB1 and malignant peripheral nerve sheath tumor.