Immunohistochemical loss of the SMARCB1/INI1 protein expression has been described in myoepithelial carcinomas and renal medullary carcinomas, in a subset of malignant peripheral nerve sheath tumors and of extraskeletal myxoid chondrosarcomas as well as in ES of both distal and proximal type. This evidence concerns the gene SMARCB1 and kidney medullary carcinoma.