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SMARCB 1/ INI 1 is one of the core subunit proteins of the  ATP ‐dependent  SWI / SNF  chromatin remodeling complex, and is identified as a potent and bona fide tumor suppressor. Interactions have been demonstrated between  SMARCB 1/ INI 1 and key proteins in various pathways related to tumor proliferation and progression: the p16‐ RB  pathway,  WNT  signaling pathway, sonic hedgehog signaling pathway and Polycomb pathway. Initially, no detectable  SMARCB 1/ INI 1 protein expression was found in malignant rhabdoid tumor cells, whereas all other kinds of tumor cells and non‐tumorous tissue showed  SMARCB 1/ INI 1 protein expression. Therefore, immunohistochemical testing for the  SMARCB 1/ INI 1 antibody has been considered useful in confirming the histologic diagnosis of malignant rhabdoid tumors. However, recently, aberrant expression of  SMARCB 1/ INI 1 has been found in various tumors such as epithelioid sarcomas, schwannomatosis, synovial sarcomas, and so on. In addition, it has been reported that aberrant expression can be classified into three patterns: complete loss, mosaic expression and reduced expression. Although the various pathways related to mechanisms of tumorigenesis and tumor proliferation are complexly intertwined, the clarification of these mechanisms may contribute to therapeutic strategies in  SMARCB 1/ INI 1‐deficient tumors. In terms of pathological classifications,  SMARCB 1/ INI 1‐deficient tumors may be re‐classified by genetic backgrounds.

Oncogenic roles of SMARCB 1/ INI 1 and its deficient tumors