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American Journal of Clinical Oncology Jul 2021Synovial sarcomas (SS) arising in distal extremities are rare and have been studied using mostly case reports and small series. We aimed to evaluate clinical...
OBJECTIVES
Synovial sarcomas (SS) arising in distal extremities are rare and have been studied using mostly case reports and small series. We aimed to evaluate clinical presentation and survival outcomes for patients with hand or foot SS.
MATERIALS AND METHODS
We conducted a retrospective review of 84 patients diagnosed with primary hand (n=20) and foot (n=64) SS between 1979 and 2019. Progression-free survival (PFS), overall survival (OS), local recurrence-free survival and metastasis-free survival were estimated using the Kaplan-Meier method and log-rank test. Cox-proportional hazards regression was used to estimate the hazard ratios.
RESULTS
Of 84 patients, 63 (75%) presented with localized disease with 36 years median age at diagnosis (range: 4 to 76) and 21 (25%) with metastasis with 30 years median age at diagnosis (range: 15 to 64). Among patients presenting with localized disease, (1) 5 years-PFS, OS, local recurrence-free survival, and metastasis-free survival rates were 82%, 88%, 100%, and 86%, respectively. (2) Tumor size <3.0 cm corresponded to 95% 5 years-PFS (vs. 84% for 3.0 to 4.9 cm, 53% for ≥5.0 cm; P=0.007) and 100% 5 years-OS (vs. 77% for ≥3.0 cm; P=0.04). (3) Patients with ≥5.0 cm (vs. <3.0 cm) tumor size had 7.99 (95% confidence interval: 1.68, 37.91) times higher hazard of progression. Remarkably, patients presenting with metastasis had 50% 5 years-OS rate. Also, younger age (15 to 39 vs. 40 y and above) predicted better OS among patients presenting with localized disease (P=0.04) and with metastasis (P=0.03).
CONCLUSIONS
Survival outcomes are favorable for younger patients with <3.0 cm hand or foot SS. Local control is excellent, but we observed larger tumor size to be associated with poorer outcomes. Therefore, we recommend consideration of systemic therapy for patients with ≥3.0 cm hand or foot SS.
Topics: Adolescent; Adult; Aged; Antineoplastic Agents; Child; Child, Preschool; Female; Foot; Hand; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Progression-Free Survival; Retrospective Studies; Sarcoma, Synovial; Soft Tissue Neoplasms; Young Adult
PubMed: 33927134
DOI: 10.1097/COC.0000000000000822 -
Expert Opinion on Emerging Drugs Mar 2019in this review we discuss the standard of care for both pediatric and adult synovial sarcoma (SS), the prognostic differences between them, and the treatments available... (Review)
Review
in this review we discuss the standard of care for both pediatric and adult synovial sarcoma (SS), the prognostic differences between them, and the treatments available for localized and advanced diseases. We also overview the biology and the recent drugs under consideration in clinical trials on SS. Areas covered: we focus on new targeted therapies being investigated for advanced SS, especially anti-angiogenic drugs, and immunotherapy. We review all the published data and ongoing trials dedicated to SS or to soft tissue sarcoma in general, paying particular attention to the results obtained in SS patients. Expert opinion: we expect new treatment strategies to become available for SS in the near future. The ongoing and published trials on targeted therapies and immunotherapy mainly concern adult patients, but the somatic biology of pediatric SS has some similarities as in adult disease. A stronger cooperation between adult and pediatric oncologists in recent years has led to a more shared effort to find new treatment strategies for advanced SS patients, regardless of their age.
Topics: Adult; Age Factors; Angiogenesis Inhibitors; Antineoplastic Agents; Child; Drug Development; Humans; Immunotherapy; Molecular Targeted Therapy; Prognosis; Sarcoma, Synovial
PubMed: 30841761
DOI: 10.1080/14728214.2019.1591367 -
Cardiovascular Pathology : the Official... 2021Primary cardiac synovial sarcoma was an exceedingly rare tumor that less reported. The study investigated the clinicopathologic, immunohistochemical, and molecular... (Review)
Review
BACKGROUND
Primary cardiac synovial sarcoma was an exceedingly rare tumor that less reported. The study investigated the clinicopathologic, immunohistochemical, and molecular features of primary cardiac synovial sarcoma.
METHODS
A total of five cardiac synovial sarcoma cases were assessed and reviewed using H&E, immunohistochemical and fluorescence in situ hybridization staining methods. Clinicopathological data were retrospectively analyzed and followed up.
RESULTS
The cases occurred in four males and one female ranging in age from 23 to 48 years (mean, 32 years). The tumors were grossly large and solid (7.4-13.7 cm; mean 8.6 cm). Microscopically, clinical cases were biphasic (n = 2) and monophasic (n = 3) types and were diffusely immunoreactive for EMA, vimentin, and BCL-2. All cases demonstrated SS18 rearrangement by fluorescence in situ hybridization staining. Clinically, three patients died within 1 year after surgery, while one patient had bone metastasis and still carried the disease. One last patient underwent a heart transplant and survived without evidence of the disease.
CONCLUSION
Cardiac synovial sarcoma was an aggressive tumor whose differentiation may be a continuous and complex morphologic spectrum. SS18 rearrangement demonstration by fluorescence in situ hybridization was decisive in our study for differential diagnosis of cardiac synovial sarcoma and other tumors. Cardiac synovial sarcoma usually endured poor survival rates. Patients in advanced stages may undergo heart transplantation as a means of improving their survival rates.
Topics: Adult; Biomarkers, Tumor; Female; Gene Rearrangement; Genetic Predisposition to Disease; Heart Neoplasms; Heart Transplantation; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Male; Middle Aged; Phenotype; Predictive Value of Tests; Proto-Oncogene Proteins; Repressor Proteins; Retrospective Studies; Sarcoma, Synovial; Treatment Outcome; Young Adult
PubMed: 32947039
DOI: 10.1016/j.carpath.2020.107286 -
Revista de Gastroenterologia de Mexico 2016
Topics: Abdominal Neoplasms; Adult; Diagnosis, Differential; Humans; Male; Sarcoma, Synovial; Tomography, X-Ray Computed
PubMed: 27198201
DOI: 10.1016/j.rgmx.2016.03.002 -
Current Opinion in Oncology Jul 2015Synovial sarcomas are a distinct soft tissue sarcoma subtype, with a predilection for young adults. Despite its common translocation, there is substantial heterogeneity... (Review)
Review
PURPOSE OF REVIEW
Synovial sarcomas are a distinct soft tissue sarcoma subtype, with a predilection for young adults. Despite its common translocation, there is substantial heterogeneity in patient outcome. This review discusses recent developments in diagnosis, prognostication, and treatments, together with the role of targeted agents and immunotherapy in patients with synovial sarcoma.
RECENT FINDINGS
Tumor behavior of synovial sarcomas remains inexplicable and is therefore poorly predictable. Although many variables seem to contribute to and influence patient outcome, no underlying pathophysiology accounting for the variability in behavior has been unraveled. As prognosis remains poor, there is a wistful search for new therapies. In preclinical testing, several receptor tyrosine kinases have been suggested as therapeutic targets with interesting results in vitro or in vivo. However, translating interesting preclinical outcome to clinical results is difficult, to a large extent due to limited patient numbers available to participate in clinical trials.
SUMMARY
By defining predictive variables, researchers try to understand the underlying cause of this tumor's biologic behavior and develop new therapeutic targets. Owing to the minimal number of prospective studies usually with small patient numbers, the strength of improving patient outcome will be in collaborative international studies in this rare tumor type.
Topics: Adult; Aged; Humans; Molecular Targeted Therapy; Prognosis; Receptor Protein-Tyrosine Kinases; Sarcoma, Synovial; Young Adult
PubMed: 26049271
DOI: 10.1097/CCO.0000000000000198 -
Cancer Epidemiology, Biomarkers &... Jun 2020Synovial sarcoma is a rare cancer with peak incidence in the young adult period. Despite poor outcomes of this aggressive cancer, there is little epidemiologic research...
BACKGROUND
Synovial sarcoma is a rare cancer with peak incidence in the young adult period. Despite poor outcomes of this aggressive cancer, there is little epidemiologic research addressing its etiology.
METHODS
We collected birth characteristic data on synovial sarcoma cases born during 1978-2015 and diagnosed during 1988-2015 in California ( = 244), and 12,200 controls frequency-matched on year of birth. We also constructed a dataset of cancer cases in siblings of sarcoma subjects to assess familial risk.
RESULTS
In multivariable logistic regression analyses, synovial sarcoma was more frequent in Hispanics compared with non-Hispanic whites [OR, 1.48; 95% confidence interval (CI), 1.06-2.08]. Higher birth weight was a risk factor in Hispanics; each 500 g increase in birth weight was associated with a 22% increase in disease risk (OR, 1.22; 95% CI, 1.00-1.48). Also, a strong role for birth order was suggested, with highest risk for the first born (second child compared with first: OR, 0.61; 95% CI, 0.44-0.84; third or later compared with first: OR, 0.53; 95% CI, 0.36-0.77). Siblings of patients with synovial sarcoma did not display elevated cancer incidence, suggesting the low likelihood that strong familial predisposition alleles play a significant role in this disease.
CONCLUSIONS
The associations with birth weight and birth order suggest that nutritional, developmental, and environmental factors may play a role in the etiology of synovial sarcoma.
IMPACT
Further epidemiologic research on synovial sarcoma should evaluate epigenetic and developmental mechanisms and the formation of the archetypical t(X;18) translocation that defines this disease.
Topics: Adolescent; Adult; Birth Order; Child; Child, Preschool; Humans; Infant; Infant, Newborn; Male; Risk Factors; Sarcoma, Synovial; Young Adult
PubMed: 32245786
DOI: 10.1158/1055-9965.EPI-20-0093 -
Current Urology Reports Mar 2021To update epidemiological, diagnostic, and therapeutic information on primary synovial sarcoma of the kidney.
PURPOSE OF REVIEW
To update epidemiological, diagnostic, and therapeutic information on primary synovial sarcoma of the kidney.
RECENT FINDINGS
A total of 96 studies were analyzed; age at presentation was 38.6±14.2 years, predominant location of tumor was right kidney; frequent reported symptoms at diagnosis were hematuria and pain. For definitive diagnosis, cytogenetic technique was used. Detected oncogene was available in 37.8% cases with fusion of SS18-SSX in most patients. Surgery is treatment of choice, with adjuvant chemotherapy; most frequently ifosfamide-based associated with doxorubicin or epirubicin. Overall median survival was 34 months. Mortality was 29% of the cases which reported death and the recurrence rate was 39.8%. Risk of death was increased in patients with metastases at diagnosis Primary RSS occurs more often in young men. RSS often presents with symptoms and in an advanced stage. Surgical treatment is the most commonly used and chemotherapy for advanced or recurrent treatment.
Topics: Hematuria; Humans; Kidney Neoplasms; Neoplasm Recurrence, Local; Oncogene Proteins, Fusion; Pain; Sarcoma, Synovial; Survival Rate
PubMed: 33704587
DOI: 10.1007/s11934-021-01038-w -
Medicina 2023Primary pericardial synovial sarcoma is an extraordinarily very rare tumor with a poor prognosis, and little is known about its therapeutic management. We describe the...
Primary pericardial synovial sarcoma is an extraordinarily very rare tumor with a poor prognosis, and little is known about its therapeutic management. We describe the case of a 51-year-old woman patient who underwent incomplete surgical resection, chemotherapy, and radiotherapy. To the best of our knowledge, no primary pericardial synovial sarcoma has been described which, after surgery, remains asymptomatic for 5 years, and until a control CT scan detects cardiac metastases that compromised the lumen of the right cavities and with chemotherapy, echocardiography demonstrated complete resolution of cardiac metastases.
Topics: Female; Humans; Middle Aged; Sarcoma, Synovial; Echocardiography; Heart Neoplasms; Thymus Neoplasms
PubMed: 37870344
DOI: No ID Found -
Cellular Oncology (Dordrecht) Jun 2022Synovial sarcoma (SySa) is a rare soft tissue tumor characterized by a reciprocal t(X;18) translocation. The chimeric SS18-SSX fusion protein represents the major driver...
PURPOSE
Synovial sarcoma (SySa) is a rare soft tissue tumor characterized by a reciprocal t(X;18) translocation. The chimeric SS18-SSX fusion protein represents the major driver of the disease, acting as aberrant transcriptional dysregulator. Oncogenic mechanisms whereby SS18-SSX mediates sarcomagenesis are incompletely understood, and strategies to selectively target SySa cells remain elusive. Based on results of Phospho-Kinase screening arrays, we here investigate the functional and therapeutic relevance of the transcription factor CREB in SySa tumorigenesis.
METHODS
Immunohistochemistry of phosphorylated CREB and its downstream targets (Rb, Cyclin D1, PCNA, Bcl-xL and Bcl-2) was performed in a large cohort of SySa. Functional aspects of CREB activity, including SS18-SSX driven circuits involved in CREB activation, were analyzed in vitro employing five SySa cell lines and a mesenchymal stem cell model. CREB mediated transcriptional activity was modulated by RNAi-mediated knockdown and small molecule inhibitors (666-15, KG-501, NASTRp and Ro 31-8220). Anti-proliferative effects of the CREB inhibitor 666-15 were tested in SySa avian chorioallantoic membrane and murine xenograft models in vivo.
RESULTS
We show that CREB is phosphorylated and activated in SySa, accompanied by downstream target expression. Human mesenchymal stem cells engineered to express SS18-SSX promote CREB expression and phosphorylation. Conversely, RNAi-mediated knockdown of SS18-SSX impairs CREB phosphorylation in SySa cells. Inhibition of CREB activity reduces downstream target expression, accompanied by suppression of SySa cell proliferation and induction of apoptosis in vitro and in vivo.
CONCLUSION
In conclusion, our data underline an essential role of CREB in SySa tumorigenesis and provides evidence for molecular targeted therapies.
Topics: Animals; Apoptosis; Carcinogenesis; Cell Line, Tumor; Humans; Mice; Oncogene Proteins, Fusion; Sarcoma, Synovial
PubMed: 35556229
DOI: 10.1007/s13402-022-00673-w -
European Journal of Surgical Oncology :... Feb 2019Synovial sarcoma, a distinct subtype of soft tissue sarcomas (STS), is typically found in young patients. Long history of symptoms and heterogeneous clinical...
OBJECTIVES
Synovial sarcoma, a distinct subtype of soft tissue sarcomas (STS), is typically found in young patients. Long history of symptoms and heterogeneous clinical presentation sometimes delays diagnosis. Children have been reported to have a better prognosis than adults in some series. The main emphasis of this study was to determine differences between children and adults and to investigate prognostic factors regarding cancer specific survival (CSS).
METHODS
248 patients treated between 1982 and 2014 at one department were included. Mean age was 37.0 years, including 43 patients <16 years. Demographic, pathology- and treatment-related information was ascertained. Median follow-up was 5.2 years.
RESULTS
Median duration of symptoms was 11.5 months in children and 12 months in adults (p = 0.238). Patients with a prior unplanned excision had a significantly longer duration of symptoms (p = 0.001). No difference was present between children and adults regarding tumour size, site, grade and superficial/deep location. Treatment was with surgical excision and (usually) adjuvant radiotherapy but five patients received preoperative radiotherapy and 43 patients chemotherapy. In patients treated with curative intent, five-year CSS rates were 75.5% for adults and 89.0% for children, with 10-year CSS rates of 56.1% and 82.2% (p = 0.026). In multivariate analysis, large tumour size (p < 0.005) and patient age (p = 0.024) were associated with worse CSS, irrespective of tumour location and site.
CONCLUSION
Clinical presentation of synovial sarcoma is similar in children and adults, with no significant difference in tumour size, site, grade or location. Small tumour size and young patient age are independent positive prognostic factors influencing CSS.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Biopsy; Child; Child, Preschool; Female; Humans; Infant; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasm Grading; Neoplasm Staging; Prognosis; Sarcoma, Synovial; Survival Analysis; Tomography, X-Ray Computed
PubMed: 30077520
DOI: 10.1016/j.ejso.2018.07.006