Birth and evolution of the desmoplastic small round-cell tumor.
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.
Department of Pathology, Institute of Biomedicine of Sevilla (IBiS), Virgen del Rocio University Hospital, CSIC, University of Sevilla, Seville 41013, Spain; Department of Pathology, La Merced Hospital, AGS Osuna, Osuna, Spain. Electronic address: enrique.alava.sspa@juntadeandalucia.es.
Enrique
E
de Alava
Department of Pathology, Institute of Biomedicine of Sevilla (IBiS), Virgen del Rocio University Hospital, CSIC, University of Sevilla, Seville 41013, Spain; Department of Pathology, La Merced Hospital, AGS Osuna, Osuna, Spain.
David
D
Marcilla
D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism; D002148:Calmodulin-Binding Proteins / Q000235:genetics / Q000378:metabolism; D003893:Desmin / Q000378:metabolism; D058405:Desmoplastic Small Round Cell Tumor / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007633:Keratins / Q000378:metabolism; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics / Q000378:metabolism; D025721:WT1 Proteins / Q000235:genetics / Q000378:metabolism
D014408:Biomarkers, Tumor; D002148:Calmodulin-Binding Proteins; D003893:Desmin; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins; D025721:WT1 Proteins; D007633:Keratins
EWSR1 gene; Molecular Pathology; Pathology; WT1 gene; desmoplasia; sarcoma