Materiały źródłowe

• #1 Synovial Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/22012-synovial-sarcoma

  Synovial sarcoma is a rare, slow-growing soft tissue cancer. The outlook is more favorable if a surgeon can remove the entire tumor before it spreads. […] Early diagnosis and treatment are helping people with synovial sarcoma live longer, with hope for a cure. […] Healthcare providers have made huge strides in treating synovial cell sarcoma. But there’s still a chance the condition will recur (return), sometimes, many years after treatment. You’ll likely have checkups for several years so your providers can monitor you closely. […] The five-year survival rate for synovial sarcoma is 50% to 60%. That means that 5 to 6 out of 10 people with synovial sarcoma are still alive five years after their diagnosis. […] The five-year metastasis-free survival rate is 40% to 60%. That means that within five years after treatment, cancer didn’t spread (metastasize) in 4 to 6 out of 10 people with synovial sarcoma.

• #2

  https://link.springer.com/article/10.1007/s11864-018-0525-1

  Synovial sarcoma (SS) is a rare, yet highly malignant, type of soft tissue sarcoma (STS), for which survival has not improved significantly during the past years. […] The prognosis of primary non metastasized SS is related to the age of the patient, with a much better relative survival in children compared to older patients, and more genomic instability with increasing age. […] The 5-year overall survival (OS) for all SS is 60.5%, but is largely age-dependent. […] Once metastasized, curative treatment is hardly achievable, with the exception of late and resectable oligometastatic disease and patients are treated with chemotherapy with a palliative intent. […] Compared to STS as a group, SS is more sensitive to certain chemotherapeutic agents. […] A recent EORTC review of 15 clinical STS trials investigated the outcome of chemotherapy in advanced SS patients, and included 313 SS patients out of 3330 STS patients.

• #3 Synovial Sarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1257131-overview

  Prognosis of this relatively rare tumor is related to initial care. Survival rates have improved in the past few decades because of treatment with primary radical surgery, along with chemotherapy and RT. […] Synovial sarcoma has been reported to be particularly likely to metastasize. Many factors modify patient outcome, such as tumor size, anatomic localization, and histologic grade. Nevertheless, histologic criteria such as nuclear grade, measures of mitotic count, and amount of necrosis are subjective and sometimes difficult to compare. […] Distal-extremity tumors have a better prognosis than proximal-extremity or truncal tumors do. […] Synovial sarcoma has survival rates of 50-60% at 5 years and 40-50% at 10 years. However, advances in oncologic therapy, particularly the development of monoclonal antibodies, may improve survival rates.

• #4 Synovial sarcoma: the importance of size and location for survival – PubMed

  https://pubmed.ncbi.nlm.nih.gov/15021147/

  A retrospective analysis was done on 135 consecutive patients with extremity and truncal synovial sarcomas seen at our institutions between 1961 and 1996. The overall survival for patients with localized primary and locally recurrent synovial sarcoma was 51% at 10 years. Patients with localized synovial sarcomas less than 5 cm in longest diameter had a survival at 10 years of 88% compared with a 10-year survival of 38% and 8% for patients with sarcomas 5 to 10 cm and greater than 10 cm in longest diameter, respectively. Distal location (hands and feet) and histologic grade also were statistically significant predictors of patient survival for localized synovial sarcoma on univariate analysis. On multivariate analysis size greater than 10 cm and size 5 to 10 cm were associated with an 18-fold and a 3.1-fold increased risk of death compared with tumors smaller than 5 cm. Primary tumor size and initial status at presentation were prognostic for survival and patients with synovial sarcomas larger than 5 cm or locally recurrent at presentation should be considered for more aggressive surgical and adjunctive radiotherapy or chemotherapy.

• #5 Age as an independent prognostic factor for survival of localised synovial sarcoma patients

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4705887/

  We show that outcome of synovial sarcoma patients significantly decreases with age regardless of primary tumour site, size, and treatment. […] The multivariable analysis identified older age, larger tumour size, and primary tumour localisation in the trunk as independent prognostic factors for worse OS in synovial sarcoma patients. […] Our study demonstrated that outcomes of patients with synovial sarcoma significantly decrease with age regardless of primary tumour site, size, and treatment.

• #6 Age as an independent prognostic factor for survival of localised synovial sarcoma patients | British Journal of Cancer

  https://www.nature.com/articles/bjc2015375

  A total of 461 patients had localised disease at diagnosis. The 5-year overall survival (OS) was 89.34.6%, 73.03.8%, 54.73.6%, and 43.07.0% in children (n=54), AYAs (n=148), adults (n=204), and elderly (n=55), respectively. […] We show that outcome of synovial sarcoma patients significantly decreases with age regardless of primary tumour site, size, and treatment. […] The multivariable analysis identified older age, larger tumour size, and primary tumour localisation in the trunk as independent prognostic factors for worse OS in synovial sarcoma patients. […] Our study demonstrated that outcomes of patients with synovial sarcoma significantly decrease with age regardless of primary tumour site, size, and treatment.

• #7 Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis

  https://www.mdpi.com/2072-6694/15/19/4860

  Synovial sarcomas are rare and highly aggressive soft-tissue sarcomas, primarily affecting adolescents and young adults aged 15–40 years. […] The 5-year survival rate for synovial sarcomas ranges from 66% to 71%. […] Notably, it is important to acknowledge that delayed local recurrences and metastases occurring more than 5 years after the initial diagnosis are common, which might necessitate extended follow-up periods. […] Numerous clinical and pathological prognostic factors have been documented. These include factors like patient age, tumor size, tumor grade, histologic subtypes, anatomical location, the effectiveness of local treatments with negative surgical margins, and the use of additional treatments such as adjuvant radiotherapy. […] Notably, patients under the age of 15 to 20 years tend to have a more favorable long-term prognosis.

• #8 Synovial Sarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1257131-overview

  A slight improvement in survival rate has been reported with the use of chemotherapy as adjuvant therapy. […] Recurrence has been reported up to 69 months after treatment and suggests a worse prognosis with low survival rates. Distant metastases at presentation suggests a bad prognosis (2-year survival rate, 25%). […] A study of 130 cases of synovial sarcoma treated by a single surgeon reported a global survival rate of 82% at the latest follow-up. […] Campbell et al described prognostic factors that correlated with a better prognosis, including the following: Biphasic histologic pattern, Patients with SYT/SSX2 fusion genes, Location in the hand or foot, Size 5 cm, Female sex, Age 50 years, Negative resection margins. […] The presence of metastases on initial presentation and tumor size exceeding 4 cm were found to be factors associated with decreased survival.

• #9 Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis

  https://www.mdpi.com/2072-6694/15/19/4860

  Among these factors, the size of the tumor at presentation, particularly tumors larger than 5 cm, significantly influences prognosis. […] Studies indicate that 5-year survival rates are 64% for patients with tumors smaller than 5 cm but only 26% for those with masses exceeding 5 cm. […] The prognosis also varies depending on the location of the tumor. Patients with tumors situated in the extremities generally have a more favorable prognosis compared to those with tumors in the head and neck region or axial region. […] Recent investigations have explored the prognostic significance of imaging features observed on CT and MRI for synovial sarcomas. […] Several imaging characteristics associated with synovial sarcomas have been linked to prognosis. […] Generally, larger tumors located more proximally and exhibiting greater complexity have correlated with worse outcomes. […] A more favorable prognosis, on the other hand, is linked to extensive calcification and tumor size below 5 cm. […] The presence of calcifications on CT scans is associated with favorable outcomes.

• #10 Prognostic Significance of Apoptosis in Synovial Sarcoma: Correlation with Clinicopathologic Parameters, Cell Proliferative Activity, and Expression of Apoptosis-Related Proteins | Modern Pathology

  https://www.nature.com/articles/3880131

  bcl-2 overexpression in synovial sarcomas has been recently reported. […] The results indicated that increased rate of apoptosis in primary synovial sarcoma was considered to be an indicator of poor prognosis. […] There was a highly significant association between high TUNEL-I value (.8%) and poor prognosis (log-rank test;P .0001). […] Univariate survival analysis by the Kaplan-Meier method and log-rank test using a TUNEL-I cutoff value of 0.8% showed that the survival rate of patients with low (0.8%) TUNEL-I values was significantly higher than that with high (0.8%) TUNEL-I values (P.0001; Fig. 2). […] In multivariate analysis, it was proved that TUNEL-I had independent prognostic value. […] In summary, the present study demonstrated that extent of apoptosis in primary synovial sarcomas correlated with that of cell proliferative activity and increased apoptosis was an indicator of poor prognosis and survival.

• #11

  https://link.springer.com/article/10.1007/s11864-018-0525-1

  The median PFS was significantly higher for SS patients compared to STS patients (6.3 vs. 3.7 months), as was the median OS (15.0 vs. 11.7 months) and the response rate (27.8 vs. 18.8%). […] In conclusion, in fit patients, data suggest that SS patients might benefit from first-line combination treatment of ifosfamide with doxorubicin over monotherapy doxorubicin, although due to the rarity of the disease this conclusion is not based on a prospective randomized study in SS. […] The role of trabectedin in SS has been investigated in a Japanese randomized phase II study comparing trabectedin to best supportive care in translocation-related sarcomas, showed a significantly better response rate (8 vs. 0%), longer median PFS (5.6 vs. 0.9 months, HR 0.07), and longer median OS (not reached vs. 8 months, HR 0.42, median follow-up 8.9 months) for the total group. […] Trabectedin in SS has never been tested in the context of a phase III trial. […] In conclusion, although it is too early to conclude on the efficacy of immunotherapy in SS, therapy based on engineered T cells showed exciting early results.

• #12 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Poor prognostic factors in adults with soft tissue sarcomas include:[4-15] […] Small low-grade tumors, particularly in the trunk or extremities, are frequently curable by surgery alone. Higher-grade sarcomas are associated with higher local-treatment failure rates and increased metastatic potential.[16] […] Prognostic nomograms (incorporating specific variables) have been developed for soft tissue sarcomas of the retroperitoneum and the extremities.[11-14] […] Late recurrences (more than 5 years from initial diagnosis) are seen with some histologies, such as synovial sarcoma or alveolar soft-part sarcoma.[17,18] […] The prognosis for patients with high-grade retroperitoneal sarcomas is less favorable than for patients with tumors at other sites, partly because of the difficulty in completely resecting these tumors and the dose-limiting toxicity of high-dose radiation therapy on visceral organs.[13-16]

• #13 Relapsed synovial sarcoma | CMAR

  https://www.dovepress.com/treatment-at-relapse-for-synovial-sarcoma-of-children-adolescents-and–peer-reviewed-fulltext-article-CMAR

  While the overall prognosis is generally quite satisfactory in children, adolescents and young adults with localised synovial sarcoma at first diagnosis, the outcome remains poor for patients after relapse. […] Approximately 25-32% of patients experience recurrence with a median time to relapse of 24 months. […] The 5-year post-relapse OS ranged from 30% to 46%. […] Type of recurrence influenced post-relapse survival in all the series, with patients with local recurrence having a better outcome than those with metastases. […] The finding that patients who succeeded in undergoing complete surgery had a better outcome was confirmed by the French experience. […] Therefore, complete surgical resection of a local relapse is required to achieve the best outcomes. […] The main prognostic variables can be used to estimate post-relapse survival, for the purposes of planning risk-adapted salvage protocols.

• #14 Relapsed synovial sarcoma | CMAR

  https://www.dovepress.com/treatment-at-relapse-for-synovial-sarcoma-of-children-adolescents-and–peer-reviewed-fulltext-article-CMAR

  In fact, in the subset of patients without any risk factor (only 7 out of 41 cases analysed for this purpose), 5-year OS was 85.7%; when at least one factor was present (early relapse or metastatic, secondary remission not reached), 5-year OS drastically decreased to 18.7%. […] The treatment of young patients with relapsed SS is currently challenging. A standardised approach is still lacking, and patient outcomes remain poor, although promising new therapies seem to be on the horizon.

• #15 Sarcoma Nomograms: Synovial Sarcoma Survival | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/nomograms/sarcoma/synovial_pre_op

  Our synovial sarcoma nomogram is a tool designed to predict the likelihood of surviving three years and five years after being diagnosed with synovial sarcoma, based on clinical and pathologic factors known before surgery. […] Probability of survival at three years and five years after being diagnosed with synovial sarcoma.

• #16 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Local disease control is crucial in patients with retroperitoneal sarcomas. Disease-specific mortality caused by local recurrence (without synchronous metastasis) was reported in up to 77% of patients with retroperitoneal sarcomas compared with 9% of patients with extremity or trunk sarcomas.[17] […] In the setting of distant metastasis, surgery may be associated with long-term disease-free survival (DFS) in patients with pulmonary metastasis and optimal underlying disease biology. This includes patients with a limited number of metastases and slow nodule growth who have undergone or are undergoing complete resection of the primary tumor.[24-26]

• #17 Synovial Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/22012-synovial-sarcoma

  Survival rates are only estimates and depend on several factors. Researchers base them on previous outcomes for other people diagnosed with the same condition. Survival rates can’t tell you how long you’ll live or how you’ll respond to a specific treatment. To learn more about survival rates or to find out what they mean for your situation talk to your healthcare provider.

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