title: Selected topics in the pathology of epithelioid soft tissue tumors.
abstract: Epithelioid morphology, mimicking carcinoma, is a key or defining feature of several soft tissue tumors and may be seen in a wide variety of other tumors. This review will focus on those tumors defined at least in part by their epithelioid morphology, in particular epithelioid sarcoma, epithelioid malignant peripheral nerve sheath tumor, and sclerosing epithelioid fibrosarcoma. The role of loss of the SMARCB1 tumor-suppressor gene in the pathogenesis of these epithelioid soft tissue tumors will be discussed, as will their differential diagnosis with non-mesenchymal tumors, in particular carcinoma and melanoma.
authors.affiliation: Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Andrew L
authors.initials: AL
authors.lastname: Folpe
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D048430:Cell Shape; D004268:DNA-Binding Proteins / Q000032:analysis / Q000235:genetics; D003937:Diagnosis, Differential; D015536:Down-Regulation; D015622:Epithelioid Cells / Q000737:chemistry / Q000473:pathology*; D005354:Fibrosarcoma / Q000473:pathology; D020022:Genetic Predisposition to Disease; D006801:Humans; D007150:Immunohistochemistry; D009442:Neurilemmoma / Q000473:pathology; D010641:Phenotype; D011237:Predictive Value of Tests; D011379:Prognosis; D018335:Rhabdoid Tumor / Q000473:pathology; D012509:Sarcoma / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D014157:Transcription Factors / Q000032:analysis / Q000235:genetics
chemical_list: D014408:Biomarkers, Tumor; D004268:DNA-Binding Proteins; C499336:SMARCA1 protein, human; D014157:Transcription Factors