Pericardial synovial sarcoma, a potential for misdiagnosis: clinicopathologic and molecular cytogenetic analysis of three cases with literature review

Am J Clin Pathol. 2012 Jan;137(1):142-9. doi: 10.1309/AJCP34ZVFLAUTMGL.

Abstract

Synovial sarcomas arising in unexpected locations may lead to diagnostic challenges. In this report, we describe 3 cases of synovial sarcoma that manifested clinically as primary pericardial lesions. All 3 cases occurred in men in their fourth decade. Fever, cough, chest pain, and chest distress were the most common symptoms. Histologically, 2 of the tumors were spindle cell monophasic, and 1 tumor was biphasic. By immunohistochemical studies, the tumor cells were positive for cytokeratins and epithelial membrane antigen. In addition, the tumor cells displayed focal immunoreactivity for calretinin, cytokeratin 5/6, and HBME-1, resulting in the initial interpretations of malignant mesotheliomas. None of the 3 cases were diagnosed correctly until subsequent molecular cytogenetic assays demonstrated the presence of SYT gene rearrangements. As there are overlapping morphologic features between pericardial synovial sarcoma and mesothelioma, molecular analysis is essential for differential diagnoses.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Biomarkers, Tumor / metabolism
  • Cytogenetics
  • DNA, Neoplasm / analysis
  • Diagnosis, Differential
  • Diagnostic Errors / prevention & control
  • Gene Rearrangement
  • Heart Neoplasms / diagnosis*
  • Heart Neoplasms / genetics
  • Heart Neoplasms / metabolism
  • Humans
  • In Situ Hybridization, Fluorescence*
  • Male
  • Mesothelioma / diagnosis
  • Pericardium / metabolism
  • Pericardium / pathology*
  • Proto-Oncogene Proteins / genetics
  • Proto-Oncogene Proteins / metabolism
  • Repressor Proteins / genetics
  • Repressor Proteins / metabolism
  • Sarcoma, Synovial / diagnosis*
  • Sarcoma, Synovial / genetics
  • Sarcoma, Synovial / metabolism

Substances

  • Biomarkers, Tumor
  • DNA, Neoplasm
  • Proto-Oncogene Proteins
  • Repressor Proteins
  • SS18 protein, human