Abstract
BACKGROUND: Cardiac synovial sarcoma of the heart is a rare, aggressive mesenchymal tumor with poor prognosis, since complete resection is seldom feasible. CASE PRESENTATION: A 23-year-old man was referred in cardiogenic shock. Emergency computed tomography (CT) revealed a large tumor with obstruction of the right atrium (RA) and prolapse into the right ventricle (RV). Resection and pericardial patch plasty were performed. Histology confirmed a G-3 spindle-cell sarcoma. At 21 months postoperatively, CT and cardiac magnetic resonance (MR) angiography showed a tumor emerging from the lateral wall of the superior caval vein (SCV) and the RA. The RA and SCV were completely resected and replaced with a tailored Dacron tunnel prosthesis. Histology confirmed R0 resection of a G-3 spindle-cell sarcoma. Reverse transcription-polymerase chain reaction (RT-PCR) confirmed a monophasic fibrous synovial sarcoma. Echocardiography upon discharge showed normal biventricular function. The heart was tumor-free upon PET-CT 24 months thereafter. A sudden progression with innumerable pulmonary nodules caused only minimal exertional dyspnea, and the patient received palliative monochemotherapy with ifosfamide. Thirty months after the first operation, he succumbed to hemorrhage from a brain metastasis. CONCLUSIONS: We report an unusually long postoperative period of 30 months in our patient after resection of a very large right atrial sarcoma. Early diagnosis, aggressive surgical treatment, adjunctive chemotherapy and radiotherapy affect survival. Systematic inclusion of patients in multicenter initiatives, including biobanking, is necessary. Better knowledge of genetic defects relevant to these cardiac tumors will promote accurate diagnoses and suggest novel and personalized gene-based therapies.