title: An osteoclast-rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts: reports of 6 cases of a GIST simulator.
abstract: Six cases are reported of an osteoclast-rich tumor of the gastrointestinal tract that should be segregated from GIST. Five of the cases were located in the small bowel and one in the stomach. The age of the patients ranged from 13 to 37 years. The tumors behaved aggressively, with metastases to regional lymph nodes, liver, and other intra-abdominal sites. Microscopically, the tumor cells were medium-sized, predominantly oval, relatively monomorphic, diffusely immunoreactive for S-100-protein, and negative for CD117, CD34, HMB-45, and Mart-1. They were admixed with scattered osteoclast-like, multinucleated giant cells which were S-100-protein negative and KP1-positive. One case studied cytogenetically had the karyotype 46XX t(12;22)(q13;q12). The cases here reported are interpreted as examples of a distinctive type of gastrointestinal neoplasm which shares some features with clear cell sarcoma of soft parts (melanoma of soft parts), including in one case the chromosomal translocation that is characteristically associated with that entity.
authors.affiliation: Department of Pathology, Yale University Medical School, New Haven, CT, USA.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Zambrano
authors.forename: Miguel
authors.initials: M
authors.lastname: Reyes-Mugica
authors.forename: Alessandro
authors.initials: A
authors.lastname: Franchi
authors.forename: Juan
authors.initials: J
authors.lastname: Rosai
mesh_terms: D000293:Adolescent; D000328:Adult; D018952:Antigens, CD34 / Q000378:metabolism; D000951:Antigens, Neoplasm; D014408:Biomarkers, Tumor / Q000378:metabolism*; D003937:Diagnosis, Differential; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000145:classification* / Q000235:genetics / Q000473:pathology* / Q000648:ultrastructure; D015726:Giant Cells / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007621:Karyotyping; D008207:Lymphatic Metastasis / Q000473:pathology; D058965:MART-1 Antigen; D008297:Male; D058950:Melanoma-Specific Antigens; D008854:Microscopy, Electron; D009363:Neoplasm Proteins / Q000378:metabolism; D010010:Osteoclasts / Q000473:pathology; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism; D009418:S100 Proteins / Q000378:metabolism; D018227:Sarcoma, Clear Cell / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000473:pathology
chemical_list: D018952:Antigens, CD34; D000951:Antigens, Neoplasm; D014408:Biomarkers, Tumor; D058965:MART-1 Antigen; C548667:MLANA protein, human; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins; D009418:S100 Proteins; D019009:Proto-Oncogene Proteins c-kit