Mesenchymal Tumors with EWSR1 Gene Rearrangements

Abstract

Among the various genes that can be rearranged in soft tissue neoplasms associated with nonrandom chromosomal translocations, EWSR1 is the most frequent one to partner with other genes to generate recurrent fusion genes. This leads to a spectrum of clinically and pathologically diverse mesenchymal and nonmesenchymal neoplasms, variably manifesting as small round cell, spindle cell, clear cell or adipocytic tumors, or tumors with distinctive myxoid stroma. This review summarizes the growing list of mesenchymal neoplasms that are associated with EWSR1 gene rearrangements.

Keywords: Angiomatoid fibrous histiocytoma; Clear cell sarcoma; Desmoplastic small round cell tumor; EWSR1; EWSR1–NFATC2; Myoepithelial tumor; Myxoid liposarcoma; Sarcoma.