Exceedingly rare SMARCB1/INI1-deficient tumors that occur more commonly in adults include synovial sarcomas, epithelioid malignant peripheral nerve sheath tumor, myoepithelial carcinoma, extraskeletal myxoid chondrosarcoma, chordoma, schwannomatosis, gastrointestinal stromal tumors (GIST), and ossifying fibromyxoid tumor (Table 1). Here, SMARCB1 is linked to Epithelioid Malignant Peripheral Nerve Sheath Tumor.