title: Intra-abdominal clear-cell sarcoma: a report of 3 cases, including 1 case with unusual morphological features, and review of the literature.
abstract: Clear-cell sarcoma (CCS) is a soft-tissue neoplasm that morphologically resembles cutaneous malignant melanoma but has a distinct molecular profile. Gastrointestinal and intra-abdominal CCSs are very rare. Here, the authors present 3 cases of intra-abdominal CCS and review the literature. Of these cases, 2 involved the small bowel, and 1 involved the peritoneum. Cases 1 and 3 had the characteristic CCS morphology, but case 2 was morphologically unusual and therefore difficult to diagnose. It had relatively small cells with less prominence of clear cells; many pseudoglandular structures were also present. It also showed aberrant expression of epithelial membrane antigen (EMA). The other 2 cases also involved some diagnostic uncertainty and were therefore referred to specialized centers. The authors wish to emphasize the importance of molecular studies in making a conclusive diagnosis of intra-abdominal CCS.
authors.affiliation: Department of Histopathology, The Christie NHS Foundation Trust, Manchester, UK. patrick.shenjere@christie.nhs.uk
authors.forename: Patrick
authors.initials: P
authors.lastname: Shenjere
authors.forename: Walid D
authors.initials: WD
authors.lastname: Salman
authors.forename: Mayuri
authors.initials: M
authors.lastname: Singh
authors.forename: David Chas
authors.initials: DC
authors.lastname: Mangham
authors.forename: Ann
authors.initials: A
authors.lastname: Williams
authors.forename: Brian Philip
authors.initials: BP
authors.lastname: Eyden
authors.forename: Nicholas
authors.initials: N
authors.lastname: Howard
authors.forename: Ben
authors.initials: B
authors.lastname: Knight
authors.forename: Saumitra S
authors.initials: SS
authors.lastname: Banerjee
mesh_terms: D000008:Abdominal Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism; D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000378:metabolism; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D050939:Gene Fusion; D015321:Gene Rearrangement; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007421:Intestine, Small / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D018396:Mucin-1 / Q000378:metabolism; D057089:Pathology, Molecular / Q000379:methods; D010537:Peritoneum / Q000473:pathology; D018227:Sarcoma, Clear Cell / Q000235:genetics / Q000378:metabolism / Q000556:secondary*; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism; D014178:Translocation, Genetic
chemical_list: D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D018396:Mucin-1