Intra-abdominal clear-cell sarcoma: a report of 3 cases, including 1 case with unusual morphological features, and review of the literature

Int J Surg Pathol. 2012 Aug;20(4):378-85. doi: 10.1177/1066896911425485. Epub 2011 Nov 13.

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abdominal Neoplasms / diagnosis*
  • Abdominal Neoplasms / genetics
  • Abdominal Neoplasms / metabolism
  • Adult
  • Aged
  • Biomarkers, Tumor / metabolism
  • DNA, Neoplasm / analysis
  • Female
  • Gene Fusion
  • Gene Rearrangement
  • Humans
  • In Situ Hybridization, Fluorescence
  • Intestine, Small / pathology
  • Lymph Nodes / pathology
  • Lymphatic Metastasis
  • Male
  • Middle Aged
  • Mucin-1 / metabolism
  • Pathology, Molecular / methods
  • Peritoneum / pathology
  • Sarcoma, Clear Cell / genetics
  • Sarcoma, Clear Cell / metabolism
  • Sarcoma, Clear Cell / secondary*
  • Soft Tissue Neoplasms / diagnosis*
  • Soft Tissue Neoplasms / genetics
  • Soft Tissue Neoplasms / metabolism
  • Translocation, Genetic

Substances

  • Biomarkers, Tumor
  • DNA, Neoplasm
  • Mucin-1