MRI Manifestations of Primary Intraspinal Synovial Sarcoma: A Case Report.

Synovial sarcomas represent a highly malignant form of mesenchymal tumors. The incidence of synovial sarcomas in the spinal canal is exceptionally rare and has been scarcely reported. Teaching Point: Comprehending the magnetic resonance imaging manifestations of intraspinal synovial sarcoma is important to prevent misdiagnosis.

The value of multidisciplinary team (MDT) management in the diagnosis and treatment of primary intrathoracic synovial sarcomas: a single-center experience.

BACKGROUND: Synovial sarcoma (SS) is a rare malignant soft tissue tumor. Primary intrathoracic SS is extremely rare, with limited diagnosis and treatment experiences. The aim of our study was to retrospectively study the clinicopathological characteristics, treatment and prognosis of primary intrathoracic SS and the impact of multidisciplinary team (MDT) management in diagnosis and treatment on patient prognosis. METHODS: The clinical and pathological characteristics, treatment, survival and prognosis of patients with primary intrathoracic SS admitted to the National Cancer Center from January 1999 to December 2018, as well as MDT intervention during diagnosis and treatment, were retrospectively analyzed. RESULTS: Thirteen patients were enrolled, including 7 (53.8%) males and 6 (46.3%) females, with primary intrathoracic SS in the lung (8/13, 61.5%), mediastinum (4/13, 30.8%) and pleura (1/13, 7.7%) as confirmed by morphological observation, immunohistochemical (IHC) staining and fluorescence in situ hybridization (FISH). Overall, 10/13 (76.9%) patients underwent surgery, and 6/10 (60.0%) received postoperative adjuvant therapy. Only 23.1% of patients received nonsurgical therapy. The MDT discussed and managed seven patients before and/or after surgery and one patient who did not undergo surgery. The estimated 3- and 5-year overall survival (OS) rates were 50.0% and 30.0%, respectively. Patients who were managed by an MDT had a longer median OS time than those who were not (46.0 vs. 18.0 months). Age (P=0.018), tumor location (P=0.029), and Ki-67 (P=0.020) were found to be significantly related to OS. CONCLUSIONS: Monophasic morphology and fusion gene characteristics are the main features for the diagnosis of primary intrathoracic SS. MDT management can help obtain accurate diagnoses and provide reasonable therapeutic options.

Clinical comparison of tenosynovial giant cell tumors, synovial chondromatosis, and synovial sarcoma: analysis and report of 53 cases.

BACKGROUND: Tenosynovial giant cell tumors (TGCTs), synovial chondromatosis (SC), and synovial sarcoma (SS) exhibit similarities in clinical features and histochemical characteristics, and differential diagnosis remains challenging in clinical practice. METHODS: Data were collected from the pathology database of Shanghai Ninth People's Hospital regarding patients who underwent surgery from 2010 to 2019 with histologically confirmed TGCTs, SC, and SS. Demographic and clinicopathological data of these patients were reviewed. Immunohistochemistry staining of 14 different markers was performed. Correlation analyses of the prognoses were evaluated. RESULTS: A total of 26 patients with TGCTs (8 diffuse TGCTs and 18 localized TGCTs), 16 with SC, and 11 with SS were identified. Pain was the main symptom of patients with both TGCTs and SC, while a palpable mass was the most common symptom for patients with SS. In addition to clinical features, we identified vital risk factors for disease recurrence. The mean follow-up periods were 51, 39, and 14 months for TGCTs, SC, and SS, respectively. Younger patients with diffuse TGCTs or patients with a higher neutrophil/lymphocyte ratio (NLR) displayed a significantly higher frequency of recurrence. We also plotted receiver operating characteristic (ROC) curve analysis for age and NLR. The area under the ROC curve (AUC) was calculated and demonstrated the ability to distinguish recurrent from nonrecurrent cases. In addition, higher CD163 expression was linked to recurrent diffuse TGCT cases. CONCLUSIONS: These data indicated possible characteristics of different aspects of TGCTs, SC, and SS. Further clarification and understanding of these factors will help with differential clinical diagnosis and recurrent risk assessment.

Primary pleural synovial sarcoma in an adolescent: a case report.

Primary pleural synovial sarcoma (SS) is an extremely rare entity, especially in the group of children and adolescents. Due to its rarity in this location and variable histogenesis, the diagnosis of SS is a clinical challenge. We report a new case of primary pleural SS in an adolescent. A 14-year-old boy initially referred to another facility for spontaneous right-sided chest pain and dyspnea, and was transferred to our hospital five days later for recurrent hemorrhagic pleural effusion in the right chest cavity. The items in routine haematological investigations were within normal limits, except for the lower level of eosinophil. CT scan revealed a heterogeneously enhancing solid mass occupied the upper two-thirds of the right hemithorax and massive pleural effusion in the lower half of the hemithorax. There was no evidence of hilar or mediastinal lymphadenopathy. Subsequently, an open thoracotomy was performed and the tumour was diagnosed as biphasic SS based on morphological and immunohistochemical analysis. The patient was discharged after surgical resection without complications and adjuvant chemotherapy was arranged. Currently, the patient is clinically well 6 months after surgery, with no evidence of recurrent disease. Though pleural SS is a rare anatomic subset of SS, it should be considered in the differential diagnosis of intrathoracic mass without hilar or mediastinal lymphadenopathy in adolescents, especially the cases with recurrent hemorrhagic pleural effusion.

Identification of Metastasis-Associated Biomarkers in Synovial Sarcoma Using Bioinformatics Analysis.

Synovial sarcoma (SS) is a highly aggressive soft tissue tumor with high risk of local recurrence and metastasis. However, the mechanisms underlying SS metastasis are still largely unclear. The purpose of this study is to screen metastasis-associated biomarkers in SS by integrated bioinformatics analysis. Two mRNA datasets (GSE40018 and GSE40021) were selected to analyze the differentially expressed genes (DEGs). Using the Database for Annotation, Visualization and Integrated Discovery (DAVID) and gene set enrichment analysis (GSEA), functional and pathway enrichment analyses were performed for DEGs. Then, the protein-protein interaction (PPI) network was constructed via the Search Tool for the Retrieval of Interacting Genes (STRING) database. The module analysis of the PPI network and hub genes validation were performed using Cytoscape software. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis of the hub genes were performed using WEB-based GEne SeT AnaLysis Toolkit (WebGestalt). The expression levels and survival analysis of hub genes were further assessed through Gene Expression Profiling Interactive Analysis (GEPIA) and the Kaplan-Meier plotter database. In total, 213 overlapping DEGs were identified, of which 109 were upregulated and 104 were downregulated. GO analysis revealed that the DEGs were predominantly involved in mitosis and cell division. KEGG pathways analysis demonstrated that most DEGs were significantly enriched in cell cycle pathway. GSEA revealed that the DEGs were mainly enriched in oocyte meiosis, cell cycle and DNA replication pathways. A key module was identified and 10 hub genes (CENPF, KIF11, KIF23, TTK, MKI67, TOP2A, CDC45, MELK, AURKB, and BUB1) were screened out. The expression and survival analysis disclosed that the 10 hub genes were upregulated in SS patients and could result in significantly reduced survival. Our study identified a series of metastasis-associated biomarkers involved in the progression of SS, and may provide novel therapeutic targets for SS metastasis.

Multimodal Risk-Adapted Treatment in Surgical Patients With Synovial Sarcoma: A Preoperative Nomogram-Guided Adjuvant Treatment Strategy.

Background: Synovial sarcoma is characterized by heterogeneous clinical manifestations, making it difficult to evaluate individual patients' prognoses and design personal treatment schemes. We established an effective preoperative nomogram to predict cancer-specific survival (CSS) and present a risk-adapted adjuvant treatment strategy in surgical patients with synovial sarcoma. Methods: This retrospective study included patients from the Surveillance, Epidemiology, and End Results (SEER) database who were diagnosed with synovial sarcoma between 1996 and 2015. The patients were randomly divided into training and validation groups. The predictors were selected using univariate and multivariate Cox hazards models. The nomogram performance was verified for its discriminatory ability and calibration. We further stratified the patients into different risk groups according to the nomogram scores and compared the efficacy of chemotherapy, radiotherapy, and combination of radiotherapy and chemotherapy. Results: There were 915 patients enrolled in our study, with 874 patients either alive or dead due to synovial sarcoma. We established a nomogram to predict 5-year CSS based on independent factors, including sex, age, grade, tumor size, location, and extent (all p < 0.05). Our model showed a consistently good discriminatory ability and calibration for predicting 5-year CSS in both the training (c-index = 0.78, 95% CI 0.75-0.81) and validation (c-index = 0.73, 95% CI 0.68-0.78). Based on their nomogram scores, we divided patients into 5 groups. Compared to patients without adjuvant treatment, nomogram I patients with adjuvant treatment had no improvements in 5-year CSS (100.0% vs. 100.0%), nomogram II patients had higher 5-year CSS with radiotherapy or chemotherapy (92.9% vs. 72.2%, p = 0.015), nomogram III patients had higher 5-year CSS with combination of chemotherapy and radiotherapy (70.1% vs. 47.2%, p = 0.004), nomogram IV patients had higher 5-year CSS with radiotherapy (41.3% vs. 15.6%, p = 0.015), and nomogram V patients had no improvements in 5-year CSS rates with adjuvant treatment (28.9% vs. 16.9%, p = 0.18). Conclusion: The nomogram showed a satisfactory discriminatory ability and calibration for predicting 5-year CSS in synovial sarcoma patients. Based on this nomogram, we stratified synovial sarcoma patients according to risk levels, which enabled us to provide a useful grouping scheme that can inform multimodal risk-adapted treatment in synovial sarcoma.

A giant pleural poorly differentiated synovial sarcoma (PDSS) in a 64-year-old woman.

The article presents the rare case of a 64-year-old woman, who was admitted to our thoracic surgery department with a giant tumor in a right hemithorax measuring 88 mm × 137 mm × 188 mm, revealed by a thoracic CT scan. An anterolateral thoracotomy with a radical tumor resection was performed. The final pathological diagnosis of the poorly differentiated synovial sarcoma (PDSS) was made. The adjuvant radiotherapy of 60 Gy in 30 fractions was applied postoperatively. One year after operation patient remains in good health. The literature review on pleural synovial sarcoma has been shortly presented.

Right ventricle inflow obstructing mass proven to be a synovial sarcoma.

Soft tissue sarcoma is the most common malignant cardiac tumor. The chief modes of presentation are embolization, obstruction, and arrhythmogenesis. We describe an unusual case of a 27-year-old man who presented with nausea and dyspnea on exertion. Transthoracic echocardiography and computed tomography revealed a huge mass in the right heart that extended through the inferior vena cava and right renal vein to the right kidney. The cardiac mass was resected, and an immunohistochemical analysis revealed it to be a TLE1-positive synovial sarcoma. After surgery, the patient received serial adjuvant chemotherapy. We herein describe the case with a brief review.