Birth and evolution of the desmoplastic small round-cell tumor

Abstract

The first large series of desmoplastic small round cell tumor was reported twenty-five years ago. This article reviews the original characterization of this neoplasm, and the eventual expansion of its clinical and pathological spectrum. Relevant data on its molecular features are summarized, in order to understand the search for therapeutic targets. The challenge ahead is to better know and cure this disease through the finding and validation of actionable therapeutic targets.

Keywords: EWSR1 gene; Molecular Pathology; Pathology; WT1 gene; desmoplasia; sarcoma.