title: Myoepithelial tumor of soft tissue: histology and genetics of an evolving entity.
abstract: Myoepithelial neoplasms represent a heterogeneous group of tumors of which classification is incomplete and evolving. Those of the soft tissues often form genetically distinct subgroups that differ from those arising within salivary glands. Soft-tissue myoepithelial tumors (including mixed tumors that show true glandular or ductal differentiation) exhibit a spectrum of different morphologic patterns, making them difficult to distinguish from a variety of other neoplasms. They have been increasingly shown to harbor genetic fusions involving EWSR1 and partner genes that are not seen in the well-characterized tumor classes involving EWSR1 translocations. We review the spectrum of soft-tissue myoepithelial tumors, discussing recent immunohistochemical and genetic findings and the differential diagnosis.
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital, London, UK.
authors.forename: Khin
authors.initials: K
authors.lastname: Thway
authors.forename: Cyril
authors.initials: C
authors.lastname: Fisher
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics*; D001706:Biopsy; D002148:Calmodulin-Binding Proteins / Q000235:genetics; D004268:DNA-Binding Proteins / Q000235:genetics; D003937:Diagnosis, Differential; D050939:Gene Fusion; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing; D006801:Humans; D007150:Immunohistochemistry; D009208:Myoepithelioma / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D010641:Phenotype; D011237:Predictive Value of Tests; D011379:Prognosis; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D014178:Translocation, Genetic
chemical_list: D014408:Biomarkers, Tumor; D002148:Calmodulin-Binding Proteins; D004268:DNA-Binding Proteins; C500986:EWSR1 protein, human; C104382:PLAG1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins