Previously, EWSR1 has been found to be pathogenetically rearranged in the benign hidradenoma of the skin, myoepithelioma, and hemangioma of bone [8, 9, 11, 12], in addition to its much more common involvement in malignant tumors such as the Ewing family of tumors, desmoplastic small round cell tumor, myxoid liposarcoma, and extraskeletal myxoid chondrosarcoma [13–16]. This evidence concerns the gene EWSR1 and desmoplastic small round cell tumor.