The Role of Surgery for Primary Pulmonary Synovial Sarcoma: A Population-Based Study.

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.

Whole-exome sequencing in primary pulmonary synovial sarcoma misdiagnosed as mesothelioma: A case report and literature review.

Synovial sarcoma is a highly malignant tumor that accounts for 10% of all soft tissue sarcomas. Primary pulmonary synovial sarcoma (PPSS) is extremely rare, and its prognosis is poor. A diagnosis is usually established after other primary lung malignancies or metastatic extrathoracic sarcomas have been excluded. Therefore, it is often misdiagnosed. In this study, we report the case of a 38-year-old woman who was misdiagnosed as having pleural mesothelioma and finally endured surgery to remove the tumor. The tumor showed SYT-SSX fusion transcripts and was diagnosed as PPSS after combining histopathological and immunohistochemical analyses. Finally, we determined some biomarkers through whole-exome sequencing (WES) to improve the diagnosis and treatment strategies.

Primary Pulmonary Synovial Sarcoma with Hemothorax: a Case Report.

Background: Synovial sarcoma is a rare and aggressive soft tissue malignancy most commonly arises from periarticular tissue of the extremities. Although several cases in the literature have reported different origins, primary pulmonary synovial sarcoma (PPSS) is an exceedingly rare and underrecognized entity, accounting for 0.5% of all lung malignancies. Clinical presentation includes chest pain, dyspnea, cough, and hemoptysis. The finding of hemothorax is a rare presentation and was barely reported in the literature. Due to its rarity and aggressive nature, the optimal treatment is unclear, while the mainstay remains surgical resection with chemo- and/or radiation therapy. Objective: To report a case of hemorrhagic effusion subsequently diagnosed with primary pulmonary synovial sarcoma with the main objective of enriching the literature regarding this rare malignancy. Case report: A 52-year-old male smoker with a background of coronary artery disease, hypertension, and diabetes mellitus was referred to our hospital. The patient presented with a history of chest pain, dyspnea, and massive right-sided pleural effusion. Laboratory investigations were unremarkable except for anemia. Chest x-ray showed a complete opacity on the right lower zone with right-sided pleural effusion. Thoracentesis was done and revealed hemorrhagic exudative effusion. Computed tomography (CT) scan showed a right heterogeneous lung mass compressing the medial segment of the middle lobe. Subsequently, the patient underwent bronchoscopy, which showed compression and edema on the right middle lobe bronchus with traces of blood coming from the right lower lobe. The patient underwent a right posterolateral thoracotomy, a fungating mass eroding the medial segment of the middle lobe was resected that was diagnosed as high-grade primary pulmonary synovial sarcoma. Radiotherapy was instituted. The patient died after two years due to recurrence. Conclusion: PPSS is an aggressive disease with poor prognostic outcomes, and Its presentation is almost similar to other lung malignancies. Meanwhile, there is no definitive management guideline, and most management depends on surgical resection if feasible with adjuvant chemo-radiation therapy.

Solitary primary pulmonary synovial sarcoma: A case report.

BACKGROUND: Synovial sarcoma (SS) is an uncommon and highly malignant soft tissue sarcoma in the clinic, with primary pulmonary SS (PPSS) being extremely rare. Here, we describe the clinical characteristics, diagnosis, and treatment of a solitary PPSS case confirmed via surgical resection and fluorescence in situ hybridization (FISH). CASE SUMMARY: A 33-year-old man was admitted because of intermittent coughing and hemoptysis for one month, with lung shadows observed for two years. Whole-body positron emission tomography-computed tomography (PET-CT) revealed a solitary mass in the upper lobe of the right lung, with uneven radioactivity uptake and a maximum standardized uptake value of 5.6. The greyish-yellow specimen obtained following thoracoscopic resection was covered with small multi-nodulated structures and consisted of soft tissue. Hematoxylin and eosin staining revealed spindle-shaped malignant tumor cells. Immunohistochemistry indicated these tumor cells were CD99 and BCL-2-positive. Furthermore, the FISH test revealed synovial sarcoma translocation genetic reassortment, which confirmed the diagnosis of SS. CONCLUSION: PPSS is extremely rare and tends to be misdiagnosed as many primary pulmonary diseases. PET-CT, histologic analysis, and FISH tests can be used to differentiate PPSS from other diseases. Surgical resection is regularly recommended for the treatment of solitary PPSS and is helpful for improving the prognosis.

Primary pulmonary synovial sarcoma.

Objective: Primary pulmonary synovial sarcoma (PPSS) is extremely rare. This study aims to identify the clinicopathologic and therapeutic factors determining survival in PPSS. Methods: We performed a retrospective analysis of 121 patients from the Surveillance, Epidemiology, and End Results Database as well as 12 patients from our own institution diagnosed with PPSS. Patient survival was evaluated using the Kaplan-Meier method. Results: The median survival time for 12 PPSS patients in our institution was 78 months. Postoperative chemotherapy (P = .027 for overall survival and P = .035 for disease-specific survival) was associated with superior survival, whereas pneumonectomy (P = .011 for overall survival and P = .006 for disease-specific survival) was associated with worse survival. Single lobe involvement (P = .022) and the absence of lymph node involvement (P = .045) were associated with improved disease-specific survival and overall survival, respectively. In the Surveillance, Epidemiology, and End Results Database, the median survival time was 23 months. Significantly superior survival was observed in patients with earlier American Joint Committee on Cancer stage (Ⅰ-Ⅱ) (P < .001 for both overall survival and disease-specific survival). Patients who were diagnosed within the recent decade did not achieve a better survival (P = .599 for overall survival and P = .596 for disease-specific survival). Conclusions: PPSS was aggressive with a very poor prognosis. The seventh American Joint Committee on Cancer stage might aid in predicting survival. Pneumonectomy and lymph node involvement might be associated with worse survival, whereas single lobe involvement and postoperative chemotherapy might be associated with improved survival.

Imaging findings of primary pulmonary synovial sarcoma with secondary distant metastases: A case report.

BACKGROUND: Synovial sarcoma (SS) accounting for 6%-10% of primary soft tissue malignancies mainly occurs in deep soft tissue adjacent to joints of the limbs. Primary pulmonary SS (PPSS) is rare and has a poor prognosis. Cases of secondary distant metastases of PPSS occur rarely and there is a lack of corresponding imaging reports. We summarized the imaging findings of PPSS with multiple metastases confirmed by surgery and pathology, and shared valuable information on PPSS. CASE SUMMARY: A 43-year-old female patient had a solid space occupying lesion in the right upper lobe of the lung. The results of a hemogram, erythrocyte sedimentation rate (ESR) and tumor markers were all within the normal range, tuberculin skin test (5 TU PPD) was negative (-). Chest computed tomography examination showed similar round soft tissue density in the posterior segment of the right upper lobe. Thoracoscopic-assisted wedge resection of the right upper lobe of the lung, right upper lobe resection and lymph node dissection were performed. Nine months after surgery, ultrasound examination showed multiple metastases on the chest wall and kidney. CONCLUSION: PPSS is a rare malignant lung tumor with strong invasiveness, early distant metastasis and poor prognosis. There are very few imaging reports. PPSS is often manifested as irregular tumor and calcification, and the metastases have extremely low echo on ultrasonography. Contrast-enhanced ultrasound indicates that the arterial phase of tumor metastases shows rapid centripetal high enhancement, manifested as "fast forward and fast regression".

[A Case Report of Primary Pulmonary Synovial Sarcoma with Postoperative Multiple Metastases Treated with Apatinib].

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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Primary pulmonary synovial sarcoma: A reappraisal.

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.

Primary pulmonary synovial sarcoma: a case report.

Primary pulmonary synovial sarcoma (PPSS) is a relatively rare mesenchymal tumor accounting for 0.5% of all primary lung malignancy with a highly progressive potential. We describe a case of a 52-year-old man whose chest CT-imaging revealed a heterogeneous mass (8.5 × 7.7 × 6.5 cm), with multiple nearly-circular nodules in bilateral pulmonary tissues. EBUS-TBNA and pathological H&E and immunohistochemical examination were performed subsequently, and sarcoma was suspected. Then molecular detection was used and PPSS was confirmed by the detection of the expression of SYT gene (positive) and EWSR1 gene (negative) in situ hybridization. This report shows that EBUS-TBNA combined with in situ hybridization could serve as a helpful diagnostic tool.

Primary pulmonary synovial sarcoma presenting with a large lump mass in the left upper mediastinum: A case report.

Primary pulmonary synovial sarcoma is a rare lesion that occurs in 0.5% cases of lung malignancies. Chest computed tomography (CT) reveals a heterogeneously enhancing mass in the lobe or hilum of the lungs, frequently calcified and with pleural invasion. Involvement of the mediastinum in the course of primary pulmonary synovial sarcoma, in particular detection of a large mass in the mediastinum as the sole initial imaging manifestation, is extremely rare, which may contribute to a delayed diagnosis or misdiagnosis. The present case report describes an extremely rare case of a patient with primary pulmonary synovial sarcoma presenting with a large mass in the left upper mediastinum. A 59-year-old patient was admitted to the Department of Respiratory Medicine of Taizhou People's Hospital in May 2014, complaining of a persistent cough and blood sputum for 2 weeks. Following admission, a chest CT showed a large mass in the left upper mediastinum. Thoracoscopy was performed and revealed that the left pulmonary artery was engulfed by the mass, and thus surgical resection of the tumor was abandoned. The patient was definitively diagnosed with primary pulmonary synovial sarcoma following the histopathological and immunohistochemical analysis of biopsy specimens obtained via thoracoscopy. Following the final diagnosis, the patient was transferred to the Department of Oncology for chemotherapy treatments with ifosfamide and doxorubicin. Unfortunately, no partial regression was achieved after two rounds of chemotherapy, and the patient was lost to follow-up 3 months after the diagnosis was confirmed. The present case may promote the consideration of primary pulmonary synovial sarcoma in the differential diagnosis of patients who present with a large mass in the mediastinum.

Diagnosis of a rare primary pulmonary synovial sarcoma with endobronchial ultrasound-guided transbronchial needle aspiration.

Primary pulmonary synovial sarcoma (PPSS) is a rare disease. Diagnosis is made postoperatively following resection of the tumor. We describe the case of a 39-year-old non-smoking woman whose chest imaging revealed a heterogeneous mass (5.4 cm × 4.6 cm), with soft tissue density in the right upper lobe and pleural effusion in the right hemithorax. The tumor was enhanced on a computed tomography scan, in which enlargement of the mediastinal lymph nodes compressing the adjacent superior vena cava was observed. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) was then performed, which demonstrated PPSS, subsequently confirmed by immunohistochemistry and the detection of a SYT-SSX fusion gene. We believe that a diagnostic approach of EBUS-TBNA for lung sarcoma would provide helpful information to clinicians.

Primary pulmonary synovial sarcoma: Diagnosis on squash smears.

Synovial sarcomas are rare tumors accounting for approximately 5-10% of soft tissue sarcomas. They occur predominantly in the extremities, followed by head and neck. Primary pulmonary sarcomas are very rare and comprise only 0.5% of all primary lung malignancies. The diagnosis is established only after sarcomas like primary lung malignancies, and metastatic sarcomas have been excluded. For synovial sarcomas that arise at unusual locations, a definitive diagnosis is challenging and requires the use of ancillary diagnostic procedures such as immunohistochemistry (IHC) and molecular genetic techniques for confirmation of diagnosis. We report a case of 29-year-old male who had right lower lobe lung mass. He underwent right lower lobectomy. Intraoperative squash smears revealed spindle cell sarcoma. Subsequent histopathology and IHC confirmed the diagnosis as synovial sarcoma. We report this case on account of its rarity and to emphasize the utility of intraoperative squash smears in the diagnosis of such cases, which has been under-utilized in clinical practice.

Primary synovial sarcoma of the lung: can haemothorax be the first manifestation?

Primary pulmonary synovial sarcomas represent a rare clinical entity and account for approximately 0.5% of lung malignancies. We report the case of a 30-year-old male who presented clinically with haemothorax. Imaging revealed a complex collection obscuring a multi-lobulated mass in the right lower lobe of the lung. He underwent a right thoracotomy for evacuation of collection and surgical resection of his pulmonary mass. Histological analysis confirmed a grade 3 monophasic fibrous synovial sarcoma of the lung with infiltration to adjacent pleura, causing his initial haemothorax. Postoperative period was uneventful and patient was referred to the oncology team for further management. Primary pulmonary synovial sarcoma, though rare, should remain an important differential when considering lung malignancies, as complete surgical resection is the mainstay of treatment.

Primary Pulmonary Synovial Sarcoma during Pregnancy: A Diagnostic and Therapeutic Dilemma.

Synovial sarcoma is a rare soft tissue sarcoma that typically arises in the extremities of young adults. We report a case of a 26-year-old pregnant woman with biopsy-proven primary synovial sarcoma of the lung that was treated with chemotherapy with radiographic response. This is only the third documented case of primary pulmonary synovial sarcoma occurring during pregnancy and the first case where chemotherapy was given.

A Case Report of Primary Pulmonary Synovial Sarcoma with Postoperative Multiple Metastases Treated with Apatinib / 中国肺癌杂志

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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A primary synovial sarcoma of lung.

Primary pulmonary synovial sarcoma is an extremely rare tumor. The diagnosis is established only after sarcoma like primary lung malignancies and metastatic sarcoma have been excluded. It has four subtypes: monophasic fibrous, monophasic epithelial, biphasic, and poorly differentiated subtypes, We report a case of a 55-year-old man, who complained of left-sided chest pain and shortness of breath, had a large heterogeneous mass, occupying most of left hemithorax, associated with ipsilateral pleural effusion, seen on contrast enhanced computed tomogram of thorax. Computed tomography guided tru-cut biopsy revealed spindle cell sarcoma. On immunohistochemistry, tumor cells expressed epithelial membrane antigen, CD99, bcl-2 and Calponin and were immunonegative for cytokeratin. So, final diagnosis was primary pulmonary synovial sarcoma, Primary pulmonary synovial sarcoma is a rarely reported case of malignant neoplasm of lung. Histopathology, immunohistochemistry, and cytogenetics, if possible, are essential for confirmation of its diagnosis.

CT and pathological analysis of primary pulmonary synovial sarcoma / 实用放射学杂志

Objective To explore the CT characteristics of primary pulmonary synovial sarcoma (PPSS)to improve the understanding of the disease.Methods The clinical,imaging and pathological data of 5 patients with PPSS confirmed by operation and pathology was analyzed retrospectively.4 patients underwent enhanced CT scan,and 1 patient underwent plain CT scan.STY-SSX fusion genes of 3 patients were detected by reverse transcriptase-polymerase chain reaction (RT-PCR).Results All the lesions were located in the upper lobe of the lung.Most lesions showed as round-like heterogeneous solid masses with clear edge,3 lesions showed heterogeneous enhancement,and 2 lesions showed circular enhancement with obviously enhanced parenchyma.1 lesion was with calcification,3 lesions were with ipsilateral pleural effusion,and mediastinal lymph nodes metastasis was seen in 1 case.The immunohistochemical results showed that the expressions of Vim,CD56,CD99,Bcl-2 and Ki-67 were positive.Only 1 case was EMA individually positive.SYT-SSX gene was detected in 3 cases.Conclusion The imaging findings of PPSS are relatively specific,the diagnosis mainly relies on pathological, immunohistochemical examination and SYT-SSX detection.

CT and pathological analysis of primary pulmonary synovial sarcoma / 实用放射学杂志

Objective To explore the CT characteristics of primary pulmonary synovial sarcoma (PPSS)to improve the understanding of the disease.Methods The clinical,imaging and pathological data of 5 patients with PPSS confirmed by operation and pathology was analyzed retrospectively.4 patients underwent enhanced CT scan,and 1 patient underwent plain CT scan.STY-SSX fusion genes of 3 patients were detected by reverse transcriptase-polymerase chain reaction (RT-PCR).Results All the lesions were located in the upper lobe of the lung.Most lesions showed as round-like heterogeneous solid masses with clear edge,3 lesions showed heterogeneous enhancement,and 2 lesions showed circular enhancement with obviously enhanced parenchyma.1 lesion was with calcification,3 lesions were with ipsilateral pleural effusion,and mediastinal lymph nodes metastasis was seen in 1 case.The immunohistochemical results showed that the expressions of Vim,CD56,CD99,Bcl-2 and Ki-67 were positive.Only 1 case was EMA individually positive.SYT-SSX gene was detected in 3 cases.Conclusion The imaging findings of PPSS are relatively specific,the diagnosis mainly relies on pathological, immunohistochemical examination and SYT-SSX detection.

A Case of Monophasic Fibrous Synovial Sarcoma Confirmed Primary Pulmonary Origin by 18F-FDG PET/CT / 결핵및호흡기질환

Most malignant mesenchymal tumors of the lung are metastases of a primary tumor from elsewhere in the body. A primary pulmonary synovial sarcoma is a very rare neoplasm that accounts for approximately 10% of soft tissue sarcomas and makes up only 0.5% of all primary lung malignancies. We report a case of a primary pulmonary synovial sarcoma in a 60-year old woman. In this case, a lung metastasis was excluded using 18F-FDG PET /CT imaging.