SMARCB1 and kidney medullary carcinoma: This SMARCB1-deficient family now comprises some clear nosological entities, such as epithelioid sarcomas (ES) [8–10], renal medullary carcinomas (RMC) [11], undifferentiated chordomas (UC) [12, 13], or epithelioid malignant peripheral nerve sheath tumors (eMPNST) [7]; it also encompasses less defined tumors and nosological entities that may be hard to diagnose or distinguish from aforementioned malignancies.