BACKGROUND To investigate the clinicopathologic, immunohistochemical (IHC), and molecular features of primary cardiac synovial sarcoma. METHODS A total of five cardiac synovial sarcoma cases were assessed and reviewed using H&E, IHC and fluorescence in situ hybridization (FISH) staining methods. Clinicopathological data were retrospectively analyzed and followed up. RESULTS The cases occurred in 4 males and 1 female ranging in age from 23 to 48 years (mean, 32 years). The tumors were grossly large and solid (7.4 to 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 FISH staining. Clinically, 3 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 Synovial sarcoma of the heart is an aggressive tumor with poor survival rates. Synovial sarcoma must be distinguished from similar tumors of the heart. Patients in advanced stages may undergo heart transplantation as a means of improving their survival rates.
F. Teng, Dong Chen, Yanwei Li
Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology