Primary cardiac synovial sarcoma: a clinicopathological, immunohistochemical, and molecular genetics study of five clinical cases

Cardiovasc Pathol. 2021 Jan-Feb:50:107286. doi: 10.1016/j.carpath.2020.107286. Epub 2020 Sep 16.


Background: Primary cardiac synovial sarcoma was an exceedingly rare tumor that less reported. The study investigated the clinicopathologic, immunohistochemical, and molecular features of primary cardiac synovial sarcoma.

Methods: A total of five cardiac synovial sarcoma cases were assessed and reviewed using H&E, immunohistochemical and fluorescence in situ hybridization staining methods. Clinicopathological data were retrospectively analyzed and followed up.

Results: The cases occurred in four males and one female ranging in age from 23 to 48 years (mean, 32 years). The tumors were grossly large and solid (7.4-13.7 cm; mean 8.6 cm). Microscopically, clinical cases were biphasic (n = 2) and monophasic (n = 3) types and were diffusely immunoreactive for EMA, vimentin, and BCL-2. All cases demonstrated SS18 rearrangement by fluorescence in situ hybridization staining. Clinically, three patients died within 1 year after surgery, while one patient had bone metastasis and still carried the disease. One last patient underwent a heart transplant and survived without evidence of the disease.

Conclusion: Cardiac synovial sarcoma was an aggressive tumor whose differentiation may be a continuous and complex morphologic spectrum. SS18 rearrangement demonstration by fluorescence in situ hybridization was decisive in our study for differential diagnosis of cardiac synovial sarcoma and other tumors. Cardiac synovial sarcoma usually endured poor survival rates. Patients in advanced stages may undergo heart transplantation as a means of improving their survival rates.

Keywords: Heart; Heart transplantation; Immunohistochemistry; Molecular genetics; Synovial sarcoma.

Publication types

  • Review

MeSH terms

  • Adult
  • Biomarkers, Tumor / analysis
  • Biomarkers, Tumor / genetics
  • Female
  • Gene Rearrangement
  • Genetic Predisposition to Disease
  • Heart Neoplasms* / chemistry
  • Heart Neoplasms* / genetics
  • Heart Neoplasms* / pathology
  • Heart Neoplasms* / surgery
  • Heart Transplantation
  • Humans
  • Immunohistochemistry*
  • In Situ Hybridization, Fluorescence*
  • Male
  • Middle Aged
  • Phenotype
  • Predictive Value of Tests
  • Proto-Oncogene Proteins / genetics
  • Repressor Proteins / genetics
  • Retrospective Studies
  • Sarcoma, Synovial* / chemistry
  • Sarcoma, Synovial* / genetics
  • Sarcoma, Synovial* / secondary
  • Sarcoma, Synovial* / surgery
  • Treatment Outcome
  • Young Adult


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