On one hand, this points toward a different underlying biology of these entities; on the other hand, many similarities between various SMARCB1 and/or different SMARCA4 tumors such as ATRT and small cell carcinomas of the ovary hypercalcemic type (SCCOHT) [20], ATRT and cribiform neuroepithelial tumors (CRINET) [21] as well as ATRT and SMARCB1-deficient chordomas [22] have been described. The gene discussed is SMARCB1; the disease is chordoma.