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In Vivo (Athens, Greece) 2019Pericardial synovial sarcomas (PSS) are very rare tumors, with dismal prognosis and limited data. We describe the clinical features and identify prognostic factors of... (Review)
Review
BACKGROUND
Pericardial synovial sarcomas (PSS) are very rare tumors, with dismal prognosis and limited data. We describe the clinical features and identify prognostic factors of primary PSS.
CASE REPORT
We describe the case of a 56-year-old male patient with PSS managed by the multidisciplinary team of thoracic oncology. The therapeutic plan comprised surgery, chemotherapy, stereotactic radiosurgery and targeted therapy, with excellent results.
MATERIALS AND METHODS
Data from 37 cases reported in English during the past 20 years were gathered and analyzed. PSS was found to occur at a mean age of 36±17.082 (range=13-67) years. Survival analysis was performed on 20 cases with follow-up of at least 6 months.
CONCLUSION
Only complete resection of the tumor seems to be an independent prognostic factor. To our knowledge, this is the first report on the safety and effectivity of pazopanib in PSS and may provide guidance for similar cases in the future.
Topics: Biopsy; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Male; Middle Aged; Pericardial Effusion; Pericardium; Positron-Emission Tomography; Radiosurgery; Sarcoma, Synovial
PubMed: 31471401
DOI: 10.21873/invivo.11633 -
Medicine Nov 2023Primary synovial sarcoma of the prostate is an extremely rare mesenchymal malignant soft tissue tumor with unique morphological features. Synovial sarcoma often occurs...
RATIONALE
Primary synovial sarcoma of the prostate is an extremely rare mesenchymal malignant soft tissue tumor with unique morphological features. Synovial sarcoma often occurs in the pararticular tissues of limbs in young people, but rarely occurs in prostate. Because it is very rare, it is easily misdiagnosed as benign prostatic hyperplasia or prostate cancer clinically. A case of synchronous acinar adenocarcinoma of the prostate has not been reported. In this article, we report a unique case of primary prostatic synovial sarcoma with acinar adenocarcinoma.
PATIENT CONCERNS
A 58-year-old male patient was found to have a prostate mass during physical examination. Prostate ultrasound examination showed an increase in prostate volume of 5.2 × 3.3 × 3.3 cm, mixed echo mass can be seen on the left side of the prostate, with a size of approximately 4.9 × 4.3 cm, left seminal vesicle compressed.
DIAGNOSES
Prostatic synovial sarcoma (biphasic type) combined with prostatic acinar adenocarcinoma (Gleason 3 + 3).
INTERVENTION
The patient received radical prostatectomy, followed by adjuvant chemotherapy and radiotherapy.
OUTCOME
After 2 months of follow-up, at the time of writing this article, the patient received a comprehensive treatment plan of adjuvant chemotherapy and radiotherapy for 2 months, and no recurrence or metastasis was found.
LESSONS
Primary prostatic synovial sarcoma (biphasic type) combined with prostatic acinar adenocarcinoma is a very unique and rare case, and effective treatment guidelines are not yet clear, posing new challenges to clinical treatment. Making full use of pathological and imaging examinations, early diagnosis and radical surgery combined with multidisciplinary treatment seem to be still a positive method.
Topics: Male; Humans; Adolescent; Middle Aged; Prostate; Sarcoma, Synovial; Prostatic Neoplasms; Prostatic Hyperplasia; Carcinoma, Acinar Cell
PubMed: 38013382
DOI: 10.1097/MD.0000000000036151 -
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 -
Cancer Reports (Hoboken, N.J.) Dec 2020This is a retrospective review of synovial sarcoma (SS) patients treated over the last 12 years in Western Australia (WA). SS is both chemo and radiotherapy sensitive....
BACKGROUND
This is a retrospective review of synovial sarcoma (SS) patients treated over the last 12 years in Western Australia (WA). SS is both chemo and radiotherapy sensitive. Results of trials in adjuvant chemotherapy are conflicting and there is limited support for neoadjuvant chemotherapy. The use of combined chemoradiotherapy is based on institutional preferences.
AIM
We reviewed the outcomes for SS patients treated in WA over a 12 year period focusing on patients who received neoadjuvant chemoradiotherapy (NACRT).
METHODS
Patient details including demographics, histopathology, treatment details, were obtained from the WA sarcoma database (2006-2018). Progression free survival (PFS) and overall survival (OS) were derived for whole cohort.
RESULTS
Twenty seven patients were identified with SS with equal gender incidence. Median age of the cohort was 36 (14-76) years. The most common primary site of disease was extremity (81.5%). 22/27 patients presented with only localized disease and 59.2% of these received neo-adjuvant treatment. Of those who received neoadjuvant treatment, 56.2% had NACRT, while 25.0% and 18.7% of patients had chemotherapy and radiotherapy respectively. Mesna, doxorubicin, ifosfamide, dacarbazine (MAID) was the most commonly used chemotherapy regimen as neoadjuvant or adjuvant treatment while ifosfamide (93.7%) was the most commonly used chemotherapy drug in any setting. There was no reported case of disease progression in group of patients who received NACRT apart from one patient who had oligometastatic disease at diagnosis. Median OS of the whole cohort was 38 months while median PFS was 24 months. Bone marrow toxicity was the most commonly reported high grade toxicity in NACRT group (55.5%) but there were no treatment related deaths.
CONCLUSION
NACRT is not widely adopted and treatment is based on institutional preferences, however our data shows that NACRT is a feasible therapy option. NACRT should be evaluated prospectively in a randomized trial.
Topics: Adolescent; Adult; Aged; Chemoradiotherapy; Female; Humans; Male; Middle Aged; Neoadjuvant Therapy; Retrospective Studies; Sarcoma, Synovial; Young Adult
PubMed: 32881345
DOI: 10.1002/cnr2.1268 -
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 -
Journal of Radiology Case Reports Feb 2015Synovial sarcoma is the fourth most common type of soft-tissue sarcoma (following undifferentiated pleomorphic sarcoma, liposarcoma, and rhabdomyosarcoma), and should be... (Review)
Review
Synovial sarcoma is the fourth most common type of soft-tissue sarcoma (following undifferentiated pleomorphic sarcoma, liposarcoma, and rhabdomyosarcoma), and should be considered a high-grade neoplasm with a high number of local recurrences and late metastases. Synovial sarcoma predominantly occurs in adolescents and young adults, and typically arises near the joints of the lower extremity. However, this tumor can also occur at uncommon sites such as the abdominal wall, which is illustrated in this article. Furthermore, we reviewed the available literatures on the clinical, pathological and radiological appearances, as well as the current knowledge concerning treatment options and prognosis.
Topics: Abdominal Wall; Adult; Biopsy, Large-Core Needle; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Sarcoma, Synovial; Tomography, X-Ray Computed
PubMed: 25926925
DOI: 10.3941/jrcr.v9i2.1992 -
Medicina 2023Synovial sarcoma is an unusual tumor with an incidence of 1-3 cases per million. It is more frequent in teenagers and young adults under 30. It develops anywhere, but...
INTRODUCTION
Synovial sarcoma is an unusual tumor with an incidence of 1-3 cases per million. It is more frequent in teenagers and young adults under 30. It develops anywhere, but the extremities are the most frequent place of appearance (80% extremities, 20% other locations: 8% trunk, 7% retroperitoneal/abdominal, 5% head and neck). Oncological results are different depending on the study. Survival rate free of local recurrence, survival rate free of events and global survival rate vary upon published studies. The same happens with the disease's prognostic factors.
METHODS
The objective was to analyze a group of 43 patients with diagnosis of synovial sarcoma of the extremities treated surgically and determine (1) global survival rate, (2) survival rate free of events, (3) local recurrence rate and (4) oncological risk factors.
RESULTS
The global survival rate at 2 years was 90% (IC95%: 76 - 96%) and 67% (IC95%: 49-80%) at 5 years. The survival rate free of events at 2 years was 68% (IC95% 51-80%) and 48% (IC95% 32-52%) at 5 years. The recurrence rate at 2 years was 9% (IC95% 3-25%) and 25% (IC95% 13-46%) at 5 years. The histological grade and metastatic presence were bad prognostic factors.
DISCUSSION
We can conclude that our oncological results are in line with those published in previous series and that there were two factors associated with poor prognosis.
Topics: Adolescent; Young Adult; Humans; Sarcoma, Synovial; Prognosis; Extremities; Neoplasm Recurrence, Local; Retrospective Studies; Survival Rate
PubMed: 37870331
DOI: No ID Found -
Journal of Cancer Research and... 2015Primary cardiac tumors are of rare presentation. We present a case of synovial sarcoma of the right atrium treated in our institution. An initial diagnosis of right... (Review)
Review
Primary cardiac tumors are of rare presentation. We present a case of synovial sarcoma of the right atrium treated in our institution. An initial diagnosis of right atrial myxoma was made based on clinico-radiological features. Intra-operatively, an irregular mass was found. Histopathologically, it was reported as monophasic synovial sarcoma. Immunohistochemistry was positive for S-100, B-cell lymphoma-2, MIC-2 and calretinin. Patient received adjuvant chemotherapy and is currently free of disease for 2 years and on regular follow-up.
Topics: Adult; Biomarkers, Tumor; Chemotherapy, Adjuvant; Heart Neoplasms; Humans; Male; Sarcoma, Synovial; Treatment Outcome; Ultrasonography
PubMed: 26458665
DOI: 10.4103/0973-1482.139391 -
Nature Communications Sep 2022Autologous T cells transduced to express a high affinity T-cell receptor specific to NY-ESO-1 (letetresgene autoleucel, lete-cel) show promise in the treatment of...
Autologous T cells transduced to express a high affinity T-cell receptor specific to NY-ESO-1 (letetresgene autoleucel, lete-cel) show promise in the treatment of metastatic synovial sarcoma, with 50% overall response rate. The efficacy of lete-cel treatment in 45 synovial sarcoma patients (NCT01343043) has been previously reported, however, biomarkers predictive of response and resistance remain to be better defined. This post-hoc analysis identifies associations of response to lete-cel with lymphodepleting chemotherapy regimen (LDR), product attributes, cell expansion, cytokines, and tumor gene expression. Responders have higher IL-15 levels pre-infusion (p = 0.011) and receive a higher number of transduced effector memory (CD45RA- CCR7-) CD8 + cells per kg (p = 0.039). Post-infusion, responders have increased IFNγ, IL-6, and peak cell expansion (p < 0.01, p < 0.01, and p = 0.016, respectively). Analysis of tumor samples post-treatment illustrates lete-cel infiltration and a decrease in expression of macrophage genes, suggesting remodeling of the tumor microenvironment. Here we report potential predictive and pharmacodynamic markers of lete-cel response that may inform LDR, cell dose, and strategies to enhance anticancer efficacy.
Topics: Antigens, Neoplasm; Biomarkers; CD8-Positive T-Lymphocytes; Humans; Membrane Proteins; Receptors, Antigen, T-Cell; Sarcoma, Synovial; Tumor Microenvironment
PubMed: 36075914
DOI: 10.1038/s41467-022-32491-x -
Cancer Discovery Feb 2015Oncogenesis in synovial sarcoma is driven by the chromosomal translocation t(X,18; p11,q11), which generates an in-frame fusion of the SWI/SNF subunit SS18 to the... (Review)
Review
UNLABELLED
Oncogenesis in synovial sarcoma is driven by the chromosomal translocation t(X,18; p11,q11), which generates an in-frame fusion of the SWI/SNF subunit SS18 to the C-terminal repression domains of SSX1 or SSX2. Proteomic studies have identified an integral role of SS18-SSX in the SWI/SNF complex, and provide new evidence for mistargeting of polycomb repression in synovial sarcoma. Two recent in vivo studies are highlighted, providing additional support for the importance of WNT signaling in synovial sarcoma: One used a conditional mouse model in which knockout of β-catenin prevents tumor formation, and the other used a small-molecule inhibitor of β-catenin in xenograft models.
SIGNIFICANCE
Synovial sarcoma appears to arise from still poorly characterized immature mesenchymal progenitor cells through the action of its primary oncogenic driver, the SS18-SSX fusion gene, which encodes a multifaceted disruptor of epigenetic control. The effects of SS18-SSX on polycomb-mediated gene repression and SWI/SNF chromatin remodeling have recently come into focus and may offer new insights into the basic function of these processes. A central role for deregulation of WNT-β-catenin signaling in synovial sarcoma has also been strengthened by recent in vivo studies. These new insights into the the biology of synovial sarcoma are guiding novel preclinical and clinical studies in this aggressive cancer.
Topics: Animals; Humans; Neoplasm Proteins; Sarcoma, Synovial; Translocation, Genetic
PubMed: 25614489
DOI: 10.1158/2159-8290.CD-14-1246