A case of primary pleuropulmonary synovial sarcoma treated by multidisciplinary treatment strategy including extrapleural pneumonectomy

A 39-year-old man undergoing highly active antiretroviral therapy (HAART) for HIV was referred to our hospital for an abnormal shadow on a chest radiograph. Chest computed tomography (CT) revealed a huge mass in the right pleural cavity. As a result of CT-guided percutaneous needle biopsy, synovial sarcoma, spindle cell carcinoma, and malignant mesothelioma were considered as differential diagnoses. A tumor biopsy by video-assisted thoracic surgery (VATS) for cytogenetic diagnosis revealed the presence of a SYT-SSX1 fusion genes; therefore, he was finally diagnosed with primary pleuropulmonary synovial sarcoma. The patient received chemotherapy with ifosfamide and doxorubicin, but the tumor and pleural nodules were still increasing in size after the chemotherapy. His general status was good and the disease remained localized within the right pleural cavity; therefore, right EPP was performed. Chest CT at 7 months after the operation showed a recurrent lesion extending from the diaphragm to the retroperitoneal space and recurrent lesions in the right chest wall. We performed tumor resections by thoracolaparotomy. Unfortunately, multiple disseminations including pericardial disseminations were detected 2 months after the 2nd surgery. We performed chemotherapy with gemcitabine and docetaxel, but the pericardial disseminations markedly increased. Then, we changed to chemotherapy with pazopanib, and stable disease (SD) was maintained for 3 months. However, he died of cardiac tamponade caused by the tumors 17 months after the initial surgery.