Thoracic synovial sarcoma: management of a rare tumor and prognosis, a report of 8 cases

Introduction: Synovial sarcomas account for 10% of soft tissue sarcomas, and are most often on the extremities. In 10% of cases, their development is extra-articular, but their primary thoracic seat remains extremely rare. Aims: We report the patients admitted for thoracic synovial sarcoma with emphasis on the main symptomatology, imaging, the means of diangnosis and the importance of adequate therapeutic management. Methods: We retrospectively reviewed the records of 8 patients managed between 2002 and 2020 in the thoracic and cardiovascular surgery department of Abderrahmen Mami Hospital Ariana – Tunisia. Results: There were 4 woman and 4 men, with a mean age of 28 years(15-43).As medical history:VON RECHLINGHAUSSEN disease(1case), Calf synovialosarcoma(1case) and 2 patients operated for synovial sarcoma;one mediastinal who received chemotherapy and the other at the pulmonary level with simple operatory follow-up. The most common signs were chest pain(4cases),cough(2cases), dyspnea(2cases),hemoptysis(2cases),superior vena cava syndrome(1case) and Café au lait spots (1case).Deterioration of general condition(3cases) and asymptomatic(1case).Chest X-ray showed an homogeneous opacity of the mediastinum(5cases),at right upper lobe (2 cases) multiple and bilateral pulmonary opacities(1case).CT scan and MRI revealed a voluminous tumor(>10cm)(3cases).Preoperative biopsy was performed(5cases).6 patients underwent surgery. The tumor was non-resecible(1case).The postoperative course was uneventful(6cases).Recurrence was noted(3cases).2 were reoperated. Conclusion: Thoracic synovial sarcoma is a rare tumor. Diagnosing these tumors may be difficult because of its slow progression sometimes leading to a fatal evolution.