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SpringerPlus 2015SS18-SSX (formerly called SYT-SSX) fusion gene has been established clinically as a molecular diagnostic test for synovial sarcoma, but the prognostic value of the...
SS18-SSX (formerly called SYT-SSX) fusion gene has been established clinically as a molecular diagnostic test for synovial sarcoma, but the prognostic value of the fusion gene variant for survival is controversial. The objective of this systematic review is to provide an up-to-date and unprecedented summary of the prognostic impact of SS18-SSX fusion type in synovial sarcoma. Studies evaluating SS18-SSX fusion type as a prognostic marker in synovial sarcoma were systematically searched for in MEDLINE, EMBASE, and Web of Science. Comparative analysis of the pooled hazard ratios (HR) between fusion types was carried out, in order to assess the likelihood of overall survival (OS), disease-specific survival (DSS), progression-free survival (PFS), and metastasis-free survival (MFS). A total of 10 studies comprising 902 patients with synovial sarcoma were considered for the meta-analysis. The pooled HR for eight eligible studies evaluating for OS or DSS was 1.28 (95% confidence interval: 0.81-2.00), suggesting no significant difference between SS18-SSX1 and SS18-SSX2 (P = 0.29). For seven studies which evaluated for PFS or MFS, the presence of SS18-SSX1 may indicate a lower survival probability than that of SS18-SSX2, although the effect did not reach a level of statistical significance (P = 0.09). There was no significant difference in OS or DSS between SS18-SSX1 and SS18-SSX2, but there were indications of SS18-SSX1 being an unfavorable prognostic factor of PFS or MFS. Further studies including cohorts with a longer follow-up period are needed.
PubMed: 26217552
DOI: 10.1186/s40064-015-1168-3 -
Annals of Surgical Oncology Dec 2017Isolated limb perfusion (ILP) and isolated limb infusion (ILI) have been variably used in recent years for the treatment of locally advanced or marginally resectable... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Isolated limb perfusion (ILP) and isolated limb infusion (ILI) have been variably used in recent years for the treatment of locally advanced or marginally resectable extremity soft tissue sarcomas (STSs). We performed a systematic review and meta-analysis of contemporary studies to further characterize treatment patterns and outcomes.
METHODS
PubMed was queried for articles published in or after the year 2000, in the English language, with > 10 patients, and with adequate outcome data following ILP/ILI. Descriptive aggregate statistics were performed.
RESULTS
Nineteen studies that met the inclusion criteria were identified, with a total of 1288 patients. Weighted mean patient age was 55.9 years and 52% were male. The majority underwent ILP (88%) versus 12% for ILI, and chemotherapeutic regimens used were as follows: (1) melphalan with tumor necrosis factor (TNF)-α (78%), (2) melphalan ± actinomycin (10%), and (3) other regimens (12%). Most common histologies treated were malignant fibrous histiocytoma (21%), liposarcoma (16%), synovial (11%) and leiomyosarcoma (7%). Aggregate overall response rate (ORR) post-procedure was 73.3%, with 25.8% demonstrating a complete response (CR). Similar unadjusted ORRs were noted in the melphalan treatment groups with and without TNFα (72.0 and 67.0%, respectively; p = 0.27). Grade III toxicity was observed in 15.4% of patients, and grade IV/V toxicity was observed in 6.0% of patients. Overall limb salvage rate was 73.8% and median time to local (in-field) progression ranged from 4 to 28 months (weighted median 22.1 months).
CONCLUSION
ILP and ILI for extremity STS can be safely performed with appreciable response rates and significant limb salvage rates. Further study is needed to identify optimal treatment regimens by histology.
Topics: Chemotherapy, Cancer, Regional Perfusion; Extremities; Humans; Limb Salvage; Prognosis; Sarcoma
PubMed: 29022281
DOI: 10.1245/s10434-017-6109-7 -
Journal of Gastrointestinal Cancer Dec 2014
Review
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Sarcoma, Synovial; Stomach Neoplasms; Young Adult
PubMed: 24595970
DOI: 10.1007/s12029-014-9591-1 -
Future Oncology (London, England) Dec 2020Synovial sarcoma (SS) is a rare, aggressive soft tissue sarcoma with a poor prognosis after metastasis. The objective of this study was to conduct a systematic review...
Synovial sarcoma (SS) is a rare, aggressive soft tissue sarcoma with a poor prognosis after metastasis. The objective of this study was to conduct a systematic review of the clinical evidence for therapeutic options for adults with metastatic or advanced SS. Relevant databases were searched with predefined keywords. Thirty-nine publications reported clinical data for systemic treatment and other interventions. Data on survival outcomes varied but were generally poor (progression-free survival: 1.0-7.7 months; overall survival: 6.7-29.2 months) for adults with metastatic and advanced SS. A high frequency of neutropenia with systemic treatment and low quality of life post-progression were reported. Reported evidence suggests poor outcomes in adults with metastatic and advanced SS and the need for the development of new treatment modalities.
Topics: Adult; Humans; Neoplasm Metastasis; Quality of Life; Sarcoma, Synovial
PubMed: 32851862
DOI: 10.2217/fon-2020-0575 -
World Journal of Clinical Oncology Feb 2020Despite the fact that about one third of patients with primary localized extremity soft tissue sarcoma (eSTS) will develop metastatic disease, abdominal metastases (AM)... (Review)
Review
BACKGROUND
Despite the fact that about one third of patients with primary localized extremity soft tissue sarcoma (eSTS) will develop metastatic disease, abdominal metastases (AM) and retroperitoneal metastases (RM) constitute rare events. There is no clear consensus on how to achieve follow-up on patients with primary localized eSTS following curative resection, especially regarding the surveillance of potential AM/RM.
AIM
To systematically analyse incidence, diagnosis, treatment and outcome of AM/RM in eSTS patients.
METHODS
In this systematic review, 899 studies available in PubMed and published between 2000 and 2018 were screened, identifying 17 original articles focused on AM or RM in eSTS. Article selection was based on the PRISMA guidelines, using the search terms (abdominal metastasis AND soft tissue sarcoma) and (soft tissue sarcoma metastasis abdomen). All studies published between January 1, 2000 and December 31, 2018 were screened. Further articles were identified by cross-searching article references, with the final search date being February 18, 2019. Due to limited data and the different reporting techniques used, the present review focused on descriptive analysis of the included studies.
RESULTS
Of the 17 studies included, six original articles reported on incidence ± diagnosis, therapy and outcome in AM and RM, whilst three original and eight case reports focused on diagnostic pathway, therapeutic procedures or outcomes without allowing conclusions regarding incidence of AM and RM. According to the former six studies, incidence of AM ranged from 0.9%-5.6% in patients with miscellaneous histological subtypes, and up to 12.1% in patients with myxoid liposarcoma. The most common histological subtypes that developed AM or RM were (myxoid) liposarcoma and leiomyosarcoma, but also rare subtypes such as epithelioid sarcoma, myxofibrosarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumour had been reported to develop AM/RM. Surgery for AM/RM was performed in five of eight case-reports (62.5%) and in 20.8%-100.0% of original articles. In particular, patients with hepatic metastases undergoing metastasectomy had a survival benefit compared to patients treated with chemotherapy or best supportive care (> 3 years < 6 mo).
CONCLUSION
Patients with eSTS should undergo surveillance with abdominal ultrasonography/computed tomography, or even whole-body-magnetic resonance imaging to detect AM/RM at an early stage.
PubMed: 32133276
DOI: 10.5306/wjco.v11.i2.74 -
Clinical Neurology and Neurosurgery Mar 2022Primary central nervous system (CNS) sarcomas represent a heterogeneous group of rare neoplasms with unclear etiology. Available data on clinical characteristics,... (Review)
Review
BACKGROUND
Primary central nervous system (CNS) sarcomas represent a heterogeneous group of rare neoplasms with unclear etiology. Available data on clinical characteristics, treatment strategies, and survival are scarce. We comprehensively reviewed management strategies and outcomes of primary CNS sarcomas in adults.
METHODS
PubMed, Scopus, and Cochrane were search following the PRISMA guidelines to include studies on primary CNS sarcomas in adults. Clinical features, management strategies, and survival were analyzed.
RESULTS
We included 9 studies comprising 78 patients. Primary CNS sarcomas were mostly intracranial (87.2%), frequently located in the parietal (17.9%), frontal (14.1%), and temporal (14.1%) lobes. Spinal CNS sarcomas were found in 10 patients (12.8%). The most common tumor histology were fibrosarcoma (16.7%), intracranial synovial sarcoma (12.8%), extraosseous mesenchymal chondrosarcoma (11.5%), perivascular sarcoma (11.5%), reticulum cell sarcoma (11.5%), and myeloid sarcoma (9%). Partial resection (57.7%) was preferred over complete resection (42.3%), and 43 patients (55.1%) received adjuvant treatments: radiotherapy (51.3%) and/or systemic chemotherapy (20.5%). 21 patients experienced CNS sarcomas recurrences, with a median progression-free survival of 9 months (range, 4-48). At last follow-up, 60 patients (76.9%) were dead, with a median overall survival of 9 months (0.1-396). Overall survival was significantly longer in patients with fibrosarcoma (p = 0.001).
CONCLUSION
Surgical resection coupled with adjuvant chemotherapy or radiation has historically been the cornerstone treatment for CNS sarcoma but showed poor local control and dismal survival. A better understanding of the CNS sarcoma microenvironment may favor the development of tailored strategies aimed at improving survival.
Topics: Adult; Central Nervous System; Central Nervous System Neoplasms; Chemotherapy, Adjuvant; Fibrosarcoma; Humans; Retrospective Studies; Sarcoma; Tumor Microenvironment
PubMed: 35151057
DOI: 10.1016/j.clineuro.2022.107127 -
ISRN Oncology 2014Background. The aim of this systematic review is to attempt to provide a descriptive analysis for cases of synovial sarcoma (SS) arising in the mediastinum and to... (Review)
Review
Background. The aim of this systematic review is to attempt to provide a descriptive analysis for cases of synovial sarcoma (SS) arising in the mediastinum and to analyze prognostic factors. Methods. We performed PubMed database search in July 2013. Twenty-two studies, which included 40 patients, form the basis of this review. Demographic and disease-related factors were analyzed for possible influence on survival. Findings were compared with extremity SS studies reported in literature. Results. Sixteen cases (40%) presented with locally advanced unresectable disease, 2 (5%) with metastatic disease, and 22 (55%) with localized resectable disease. Median tumor size was 11 cm (range: 5-20 cm). Thirty patients were assessable for survival and had a 5-year OS of 36%. Completeness of resection was the only factor associated with significant improvement in OS (5-year survival of 63% and 0% in favor of complete resection, P = 0.003). Conclusion. Mediastinal SS is associated with poor prognosis as more cases are diagnosed at an advanced stage and with larger tumor size compared to extremity SS. Complete surgical resection is the only identified factor associated with better prognosis and may result in survival outcomes that are comparable with those for localized SS of the extremity.
PubMed: 24563786
DOI: 10.1155/2014/412527 -
Journal of Neurosurgery. Spine Jun 2024Synovial sarcoma (SS) is a relatively rare type of soft-tissue sarcoma that is commonly treated with surgery, radiation, chemotherapy, and palliative care. Stereotactic... (Review)
Review
OBJECTIVE
Synovial sarcoma (SS) is a relatively rare type of soft-tissue sarcoma that is commonly treated with surgery, radiation, chemotherapy, and palliative care. Stereotactic radiosurgery (SRS) is an emerging approach that shows promise in treating CNS conditions, but it has not been studied for SS. The authors present a systematic review that explores the effectiveness of different treatments, with a focus on SRS, for managing spinal SS.
METHODS
A systematic PubMed search was conducted that covered studies from 1964 to 2022, yielding 70 relevant studies. Inclusion criteria encompassed primary and metastatic spinal SS, various treatment modalities, patient age 17 years or older, English-language studies, retrospective series, and case reports. Based on these criteria, 26 studies were included in this review and 44 were excluded.
RESULTS
Of the included studies, 15 patients from 9 studies were treated with surgical intervention followed by both conventional radiotherapy (RT) and chemotherapy, 10 patients from 10 studies were treated with surgery followed by RT, 5 studies comprising 8 patients were exclusively treated with surgery, 5 cases in 3 studies were treated with surgery plus concomitant chemotherapy, 4 patients in 2 studies were treated with SRS, and only 1 study reported treatment without surgery and with chemotherapy and RT. The median progression-free survival and overall survival periods observed in the SRS-treated patients were 37 months and 60 months, respectively, which were higher than those of any other treatment method or combination used.
CONCLUSIONS
The authors' study offers a thorough review of spinal SS treatments. They are hopeful that this will aid clinicians in informed decision-making for better patient outcomes.
Topics: Humans; Sarcoma, Synovial; Spinal Neoplasms; Radiosurgery; Combined Modality Therapy
PubMed: 38489819
DOI: 10.3171/2024.1.SPINE231184 -
Cancers Oct 2022The aim of this study was to systematically review the literature of sarcoma of the parotid gland in order to analyze the main factors affecting survival rate. A... (Review)
Review
Systematic Review of Parotid Gland Sarcomas: Multi-Variate Analysis of Clinicopathologic Findings, Therapeutic Approaches and Oncological Outcomes That Affect Survival Rate.
The aim of this study was to systematically review the literature of sarcoma of the parotid gland in order to analyze the main factors affecting survival rate. A systematic literature review was performed between January 1990 to November 2021, and 88 patients affected by parotid gland sarcomas were included. The most common histological types were Rhabdomyosarcoma and Synovial Sarcoma. From our review, it emerges that primary sarcomas of the parotid glands are locally aggressive but show low tendency to metastasize to the lymph nodes of the neck and that surgery (i.e., total or radical parotidectomy) is the main approach for their treatment. The global overall survival (OS) is 52% at 5 years and 34.1% at 10 years. The OS for T1, T2, T3, T4 tumor at 5 years of follow up is 80.0%, 66.5%, 56.7% and 33.3%, respectively. Size/extension at the diagnosis and the sarcoma's histotype are the most important prognostic factors. Multivariate analysis showed that surgery (total or radical parotidectomy) performed on the tumor ( = 0.0008) was the only parameter that significantly affected the OS. Among the other variables, age (younger), use of adjuvant therapy and lymph node metastasis showed borderline significative values ( = 0.05). Our analysis suggests that, when a primitive parotid sarcoma is diagnosed, total or radical parotidectomy should be performed at any age independent of tumor histology. Because regional lymph node metastases from parotid sarcomas are uncommon, alternative strategies (e.g., close follow-up by imaging and evaluation of sentinel lymph nodes) should be pursued before lymph node (selective/radical) dissection.
PubMed: 36230786
DOI: 10.3390/cancers14194862 -
Journal of Gastrointestinal Cancer Mar 2019
Diffuse, Aggressive Metastatic Progression after Minimally Invasive Local Resection of Primary Gastric Synovial Sarcoma: a Case Report and Systematic Review of the Literature.
Topics: Adult; Aged; Disease Progression; Female; Humans; Male; Middle Aged; Neoplasm Metastasis; Sarcoma, Synovial; Stomach Neoplasms; Young Adult
PubMed: 28660525
DOI: 10.1007/s12029-017-9979-9