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Introduction: The rarity of primary pulmonary synovial sarcoma (PPSS) and the lack of prospective clinical trials resulted in poorly understood treatment modality and clinical outcomes. This study aimed to better understand PPSS based on patients from the Surveillance, Epidemiology, and End Results database., Materials and Methods: Clinical and survival data of PPSS patients who were diagnosed during 1989 through 2016 and retrieved from the Surveillance, Epidemiology, and End Results database were studied. Kaplan-Meier analyses and Cox proportional hazards model were applied to evaluate the overall survival (OS) and disease-specific survival (DSS) of PPSS patients., Results: A total of 122 patients with PPSS were included (median age: 50 y). PPSS accounted for 4.5% (122/2741) of total primary synovial sarcoma. Most of the patients were diagnosed as poor or undifferentiated grade (52.0% and 34.0%). Cancer-directed surgery was performed for 74.4% of PPSS patients and 28.2% of patients received radiotherapy. The 1-year, 3-year, 5-year, and 10-year OS rates of PPSS patients were 75.4%, 50.8%, 41.8%, and 39.3%, respectively. Cancer-directed surgery was shown to improve the survival of PPSS patients with localized or regional stage (P < 0.05), yet surgical resection did not prolong the OS and DSS of patients with distant stage (P > 0.1). Postoperational radiotherapy was associated with shortened survival time (P < 0.05). PPSS patients who received lobectomy had statistically prolonged OS and DSS than those with pneumonectomy (P < 0.001)., Conclusions: PPSS is a rare and special subtype of synovial sarcoma. Treatment with lobectomy or sublobar resection alone may contribute to a superior prognosis compared with other managements., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Primary pulmonary synovial sarcoma is a rare pulmonary malignant tumor originated from primitive mesenchymal, which has short overall survival and poor prognosis. Related case reports are lacked at home and abroad. In recent years, the development of targeted therapy has brought remarkable benefits to cancer patients. Apatinib (Hengrui Pharmaceutical Co. Ltd, Jiangsu, People's Republic of China) is a small molecule vascular endothelial growth factor receptor 2 (VEGFR-2) inhibitor, which selectively inhibits VEGFR-2 and blocks the VEGF signal pathway, then strongly inhibiting the tumor angiogenesis. Apatinib has shown favorable therapeutic effect and acceptable toxicity on various tumors. Here we report a case of primary pulmonary synovial sarcoma with postoperative multiple metastases treated with apatinib, in order to provide a new considerable treatment.
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Synovial sarcoma (SS) is a malignant mesenchymal tumor with variable epithelial differentiation that affects mostly young adults and can arise at any anatomic site. Primary intrathoracic SS is very rare accounting for <0.5% of all lung tumors. Most commonly, it arises from the lung followed by pleura and mediastinum. Primary pulmonary SS (PPSS) affects both sexes equally with no preference for any hemithorax. The morphology, immunostaining properties, cytogenetic features, and management strategy of PPSS are similar to that of soft tissue SS. Histologically, there are two main types of SS - monophasic and biphasic with a feature of poor differentiation seen in both types. Most patients present with large intrathoracic masses with or without ipsilateral pleural effusion. Bone invasion or mediastinal adenopathy is very rare. SS is characterized by a specific chromosomal translocation producing SS18-SSX fusion gene in more than 90% of cases. Identification of this fusion gene remains the gold standard for the diagnosis in the presence of consistent histology and immunophenotype. Multimodality treatment including wide excision, chemotherapy, and radiotherapy is the mainstay of therapy. SS is relatively chemosensitive, and ifosfamide-based regimen showed improved survival in metastatic disease. Generally, SS is considered as high-grade tumors with a poor prognosis. Novel therapies targeted at fusion oncogene, SS18-SSX-derived peptide vaccine, epidermal growth factor receptor, and vascular endothelial growth factor are the future hope in SS. We describe a prototype case and present an elaborate review on primary SS of lung., Competing Interests: There are no conflicts of interest

Primary pulmonary synovial sarcoma is a rare pulmonary malignant tumor originated from primitive mesenchymal, which has short overall survival and poor prognosis. Related case reports are lacked at home and abroad. In recent years, the development of targeted therapy has brought remarkable benefits to cancer patients. Apatinib (Hengrui Pharmaceutical Co. Ltd, Jiangsu, People’s Republic of China) is a small molecule vascular endothelial growth factor receptor 2 (VEGFR-2) inhibitor, which selectively inhibits VEGFR-2 and blocks the VEGF signal pathway, then strongly inhibiting the tumor angiogenesis. Apatinib has shown favorable therapeutic effect and acceptable toxicity on various tumors. Here we report a case of primary pulmonary synovial sarcoma with postoperative multiple metastases treated with apatinib, in order to provide a new considerable treatment.