title: Clear cell sarcoma-like tumor with osteoclast-like giant cells in the small bowel: further evidence for a new tumor entity.
abstract: Most mesenchymal neoplasms of the gastrointestinal tract belong to the category of gastrointestinal stromal tumors (GISTs) and are characterized by the immunohistochemical expression of KIT receptor. In cases without detectable KIT receptor expression several differential diagnoses have to be taken into consideration. Here, we report a case of a 41-year-old man with a tumor of the small bowel composed of large epithelioid tumor cells arranged in solid and alveolar sheets including scattered osteoclast-like multinucleated giant cells. Immunohistochemically, the tumor cells expressed strongly S-100 protein, vimentin, and to a lesser extent, bcl-2. HMB-45, melan-A, KIT receptor, desmin, smooth-muscle actin, and CD-34 were not detectable. Ki-67 index was 20%. The diagnosis was established by 2 different FISH strategies demostrating the presence of a t(12;22)(q13;q12) translocation, the diagnostic hallmark of clear cell sarcoma of soft parts. Our results provide further evidence for the existence of a new tumor entity designated gastrointestinal clear cell sarcoma with osteoclast-like giant cells. The diagnosis of this entity should be considered in the presence of S-100-positive tumors of the gastrointestinal tract containing multinucleated giant cells and can be established by FISH analysis.
authors.affiliation: Department of Pathology, University of Bonn Medical School, Bonn, Germany.
authors.forename: Nicolaus
authors.initials: N
authors.lastname: Friedrichs
authors.forename: Maria Adele
authors.initials: MA
authors.lastname: Testi
authors.forename: Luisa
authors.initials: L
authors.lastname: Moiraghi
authors.forename: Piergiorgio
authors.initials: P
authors.lastname: Modena
authors.forename: Ellen
authors.initials: E
authors.lastname: Paggen
authors.forename: Andreas
authors.initials: A
authors.lastname: Plötner
authors.forename: Volker
authors.initials: V
authors.lastname: Wiechmann
authors.forename: Luisa
authors.initials: L
authors.lastname: Mantovani-Löffler
authors.forename: Sabine
authors.initials: S
authors.lastname: Merkelbach-Bruse
authors.forename: Reinhard
authors.initials: R
authors.lastname: Buettner
authors.forename: Eva
authors.initials: E
authors.lastname: Wardelmann
mesh_terms: D000328:Adult; D000951:Antigens, Neoplasm; D002881:Chromosomes, Human, Pair 12; D002892:Chromosomes, Human, Pair 22; D003937:Diagnosis, Differential; D015726:Giant Cells / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007580:Jejunal Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D008297:Male; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins / Q000032:analysis; D010010:Osteoclasts / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D020800:Receptor, Nerve Growth Factor / Q000032:analysis; D009418:S100 Proteins / Q000032:analysis; D018227:Sarcoma, Clear Cell / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D014178:Translocation, Genetic; D014746:Vimentin / Q000032:analysis
chemical_list: D000951:Antigens, Neoplasm; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins; D020800:Receptor, Nerve Growth Factor; D009418:S100 Proteins; D014746:Vimentin; D010751:Phosphopyruvate Hydratase