Specifically, the core subunit SMARCB1 (INI1), is the most frequently inactivated subunit in mesenchymal neoplasms [7], with SMARCB1 deficiency being characteristic of malignant rhabdoid tumors (MRT), epithelioid sarcoma (ES), and most of poorly differentiated chordoma. The gene discussed is SMARCB1; the disease is epithelioid sarcoma.