title: Intestinal clear cell sarcoma with melanocytic differentiation and EWS [corrected] rearrangement: report of a case.
abstract: A clear cell sarcoma-like tumor with osteoclast-like giant cells of the gastrointestinal tract without immunoexpression of CD117 was recently proposed as a new tumor entity. In this article, a case of a 37-year-old man with a neoplasm of the jejunum composed of polygonal cells with clear to eosinophilic cytoplasm forming nests and fascicles is reported. Giant cells were not identified. Immunohistochemically, the tumor cells expressed strongly S100 protein, human melanoma black 45, platelet-derived growth factor receptor alpha, B-cell lymphoma 2, p53, and to a lesser extent vimentin, neuron-specific enolase, and epithelial membrane antigen. Mindbomb homolog-1 index was 35%. Immunoreactivity for CD34 and CD117 was negative. The fluorescence in situ hybridization analysis showed a translocation involving chromosome 22q12, the diagnostic hallmark of clear cell sarcoma of soft tissues. This case indicates a close histogenetic relationship with the recently reported clear cell sarcoma with osteoclast-like giant cells of the gastrointestinal tract, as well as with the clear cell sarcoma of soft tissues.
authors.affiliation: Department of Pathology, Hospital Clínico Universitario, SERGAS, University of Santiago de Compostela, Galicia, Spain. ihab.abdulkader.nallib@sergas.es
authors.forename: Ihab
authors.initials: I
authors.lastname: Abdulkader
authors.forename: José
authors.initials: J
authors.lastname: Cameselle-Teijeiro
authors.forename: Enrique
authors.initials: E
authors.lastname: de Alava
authors.forename: Clara
authors.initials: C
authors.lastname: Ruiz-Ponte
authors.forename: María M
authors.initials: MM
authors.lastname: Used-Aznar
authors.forename: Jerónimo
authors.initials: J
authors.lastname: Forteza
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D002148:Calmodulin-Binding Proteins / Q000235:genetics; D002881:Chromosomes, Human, Pair 12; D002892:Chromosomes, Human, Pair 22; D003131:Combined Modality Therapy; D004268:DNA-Binding Proteins / Q000032:analysis / Q000235:genetics; D006801:Humans; D007580:Jejunal Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D008297:Male; D008543:Melanins / Q000032:analysis; D008544:Melanocytes / Q000737:chemistry / Q000473:pathology*; D009687:Nuclear Proteins / Q000032:analysis / Q000235:genetics; D010010:Osteoclasts / Q000737:chemistry / Q000473:pathology; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics; D000071701:Regulatory Factor X Transcription Factors; D018227:Sarcoma, Clear Cell / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D014157:Transcription Factors; D014178:Translocation, Genetic / Q000235:genetics
chemical_list: D014408:Biomarkers, Tumor; D002148:Calmodulin-Binding Proteins; D004268:DNA-Binding Proteins; C500986:EWSR1 protein, human; D008543:Melanins; D009687:Nuclear Proteins; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins; D000071701:Regulatory Factor X Transcription Factors; D014157:Transcription Factors