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Current Treatment Options in Oncology Oct 2021New molecular insights are being achieved in synovial sarcoma (SS) that can provide new potential diagnostic and prognostic markers as well as therapeutic targets. In... (Review)
Review
New molecular insights are being achieved in synovial sarcoma (SS) that can provide new potential diagnostic and prognostic markers as well as therapeutic targets. In particular, the advancement of research on epigenomics and gene regulation is promising. The concrete hypothesis that the pathogenesis of SS might mainly depend on the disruption of the balance of the complex interaction between epigenomic regulatory complexes and the consequences on gene expression opens interesting new perspectives. The standard of care for primary SS is wide surgical resection combined with radiation in selected cases. The role of chemotherapy is still under refinement and can be considered in patients at high risk of metastasis or in those with advanced disease. Cytotoxic chemotherapy (anthracyclines, ifosfamide, trabectedin, and pazopanib) is the treatment of choice, despite several possible side effects. Many possible drug-able targets have been identified. However, the impact of these strategies in improving SS outcome is still limited, thus making current and future research strongly needed to improve the survival of patients with SS.
Topics: Anthracyclines; Antineoplastic Agents; Chemotherapy, Adjuvant; Epigenomics; Gene Expression Regulation, Neoplastic; Genomics; Humans; Ifosfamide; Indazoles; Molecular Targeted Therapy; Pyrimidines; Radiotherapy, Adjuvant; Sarcoma, Synovial; Soft Tissue Neoplasms; Sulfonamides; Surgical Procedures, Operative; Trabectedin
PubMed: 34687366
DOI: 10.1007/s11864-021-00914-4 -
Annals of Diagnostic Pathology Dec 2014Synovial sarcoma (SS) is a malignant mesenchymal neoplasm with variable epithelial differentiation, with a propensity to occur in young adults and which can arise at... (Review)
Review
Synovial sarcoma (SS) is a malignant mesenchymal neoplasm with variable epithelial differentiation, with a propensity to occur in young adults and which can arise at almost any site. It is generally viewed and treated as a high-grade sarcoma. As one of the first sarcomas to be defined by the presence of a specific chromosomal translocation leading to the production of the SS18-SSX fusion oncogene, it is perhaps the archetypal "translocation-associated sarcoma," and its translocation remains unique to this tumor type. Synovial sarcoma has a variety of morphologic patterns, but its chief forms are the classic biphasic pattern, of glandular or solid epithelial structures with monomorphic spindle cells and the monophasic pattern, of fascicles of spindle cells with only immunohistochemical or ultrastructural evidence of epithelial differentiation. However, there is significant morphologic heterogeneity and overlap with a variety of other neoplasms, which can cause diagnostic challenge, particularly as the immunoprofile is varied, SS18-SSX is not detected in 100% of SSs, and they may occur at unusual sites. Correct diagnosis is clinically important, due to the relative chemosensitivity of SS in relation to other sarcomas, for prognostication and because of the potential for treatment with specific targeted therapies in the near future. We review SS, with emphasis on the diagnostic spectrum, recent immunohistochemical and genetic findings, and the differential diagnosis.
Topics: Biomarkers, Tumor; Cell Differentiation; Diagnosis, Differential; Epithelial Cells; Humans; Oncogene Proteins, Fusion; Sarcoma, Synovial
PubMed: 25438927
DOI: 10.1016/j.anndiagpath.2014.09.002 -
Southern Medical Journal Jan 2020The purpose of this study was to investigate the patient population and outcomes of synovial sarcoma at a single institution.
OBJECTIVES
The purpose of this study was to investigate the patient population and outcomes of synovial sarcoma at a single institution.
METHODS
A retrospective review of the medical records of 28 patients with synovial sarcoma diagnosed from 1992 to 2017 was performed. Demographics, staging, disease location, treatment, and response to treatment were reviewed.
RESULTS
Individuals with larger tumors at the time of presentation had an increased risk of death. An additional factor associated with poor prognosis in synovial sarcoma was increasing patient age. The patient population had a higher rate of nonextremity disease and lower overall survival when compared with national averages.
CONCLUSIONS
Nonextremity disease and large size of tumor at presentation may have contributed to the disparity in institutional outcomes from the national averages. The advanced presentation of synovial sarcoma remains a significant challenge in improving patient survival.
Topics: Adult; Age Factors; Female; Health Status Disparities; Humans; Male; Neoplasm Staging; Prognosis; Retrospective Studies; Sarcoma, Synovial; Survival Rate
PubMed: 31897493
DOI: 10.14423/SMJ.0000000000001054 -
Current Oncology Reports Oct 2020Aside from a characteristic SS18-SSX translocation identified in almost all cases, no genetic anomalies have been reliably isolated yet to drive the pathogenesis of... (Review)
Review
PURPOSE OF REVIEW
Aside from a characteristic SS18-SSX translocation identified in almost all cases, no genetic anomalies have been reliably isolated yet to drive the pathogenesis of synovial sarcoma. In the following review, we explore the structural units of wild-type SS18 and SSX, particularly as they relate to the transcriptional alterations and cellular pathway changes imposed by SS18-SSX.
RECENT FINDINGS
Native SS18 and SSX contribute recognizable domains to the SS18-SSX chimeric proteins, which inflict transcriptional and epigenetic changes through selective protein interactions involving the SWI/SNF and Polycomb chromatin remodeling complexes. Multiple oncogenic and developmental pathways become altered, collectively reprogramming the cellular origin of synovial sarcoma and promoting its malignant transformation. Synovial sarcoma is characterized by complex epigenetic and signaling landscapes. Identifying the operational pathways and concomitant genetic changes induced by SS18-SSX fusions could help develop tailored therapeutic strategies to ultimately improve disease control and patient survivorship.
Topics: Epigenesis, Genetic; Humans; Oncogene Proteins, Fusion; Sarcoma, Synovial; Signal Transduction; Translocation, Genetic
PubMed: 33025259
DOI: 10.1007/s11912-020-00985-w -
Retinal Cases & Brief ReportsTo describe the first reported case of intraocular synovial sarcoma.
PURPOSE
To describe the first reported case of intraocular synovial sarcoma.
METHODS
A 29-year-old man was enucleated for a blind, painful eye. Pathologic examination revealed an unexpected intraocular spindle cell tumor.
RESULTS
Immunohistochemical characterization revealed diffuse reactivity of the tumor cells for vimentin and focal positivity for epithelial markers pankeratin and epithelial membrane antigen. Melanoma markers were negative. Fluorescent in situ hybridization studies identified a t(X;18) (p11.2;q11.2) translocation, establishing a final diagnosis of synovial sarcoma. There was no evidence of extraocular extension as the resected margin of the optic nerve was free of tumor. Further imaging studies revealed no extraocular primary site or metastasis.
CONCLUSION
The incidental discovery of an intraocular malignancy in this case underscores the importance of routine histopathologic analysis of all enucleated globes. To the authors' knowledge, this is the first reported case of an intraocular synovial sarcoma, either as metastasis or as primary site.
Topics: Adult; Eye Neoplasms; Humans; Male; Sarcoma, Synovial
PubMed: 27315324
DOI: 10.1097/ICB.0000000000000348 -
Current Treatment Options in Oncology Mar 2018Synovial 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. In... (Review)
Review
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. In this review, we focus on systemic treatment in adults. Compared to other STS, SS are relatively chemosensitive. Ifosfamide and ifosfamide combinations are active in different lines of treatment. In high-risk extremity and chest wall STS, neoadjuvant doxorubicin and ifosfamide has shown as much activity as high-dose ifosfamide. There are indications that combination chemotherapy with doxorubicin and ifosfamide in this setting improves outcome. In the first-line metastatic setting, combination treatment with doxorubicin and ifosfamide is a preferred option in fit patients, while in other patients, sequential doxorubicin and ifosfamide can be considered. In second and later lines, pazopanib and trabectedin have shown activity. Many new approaches to treat metastatic SS are currently under investigation, both preclinical as well as clinical, including other receptor tyrosine kinase inhibitors, epigenetic modulators, compounds interfering with DNA damage response (DDR), and immunotherapy.
Topics: Adult; Antineoplastic Agents; Antineoplastic Combined Chemotherapy Protocols; Doxorubicin; Humans; Ifosfamide; Indazoles; Pyrimidines; Sarcoma, Synovial; Sulfonamides; Trabectedin
PubMed: 29516254
DOI: 10.1007/s11864-018-0525-1 -
ANZ Journal of Surgery Apr 2021
Topics: Esophagus; Humans; Sarcoma, Synovial
PubMed: 32857866
DOI: 10.1111/ans.16237 -
BMJ Case Reports Nov 2021Synovial sarcoma (SS) has a rare occurrence in the female genital tract. Only three prior reports of primary ovarian sarcoma could be retrieved after a thorough...
Synovial sarcoma (SS) has a rare occurrence in the female genital tract. Only three prior reports of primary ovarian sarcoma could be retrieved after a thorough literature review. We are reporting a case of primary ovarian SS in a young woman. The tumour showed monophasic spindle cell morphology, and there was a wide list of differential diagnosis to consider. We confirmed the diagnosis by cytogenetics Flourescent Insitu Hybridisation (FISH) technique to identify the classical translocation. The diagnosis of this disease can be challenging especially if the tumour is of monophasic type. Morphology and immunohistochemistry are not enough to confirm the diagnosis in many cases. A confirmatory molecular pathology test is paramount. We have discussed the differential diagnosis of spindle cell tumours in ovary. We suggest that SS should be in the differential diagnoses when facing any atypical spindle cell tumour in the ovary. Molecular pathology techniques can help to confirm the diagnosis.
Topics: Female; Humans; Immunohistochemistry; Ovarian Neoplasms; Ovary; Sarcoma, Synovial; Translocation, Genetic
PubMed: 34753724
DOI: 10.1136/bcr-2021-244756 -
JBJS Reviews Dec 2023» Synovial sarcoma is a soft tissue sarcoma that most commonly presents in the extremity in a periarticular location.» As the history and physical examination of...
» Synovial sarcoma is a soft tissue sarcoma that most commonly presents in the extremity in a periarticular location.» As the history and physical examination of patients with synovial sarcoma can overlap considerably with those of patients with non-oncologic orthopedic conditions, it is important that orthopedic surgeons maintain a high level of suspicion when caring for patients with extremity masses.» Soft tissue sarcomas are best treated using a team approach. Early recognition and referral to a multidisciplinary sarcoma team are crucial to ensure the best clinical outcome for the patient.
Topics: Humans; Sarcoma, Synovial; Extremities; Sarcoma; Soft Tissue Neoplasms
PubMed: 38117909
DOI: 10.2106/JBJS.RVW.23.00171 -
Modern Pathology : An Official Journal... Jan 2024We report the clinicopathologic and immunohistochemical features of 18 cases of confirmed primary synovial sarcoma of the gastrointestinal tract. The neoplasms arose in...
We report the clinicopathologic and immunohistochemical features of 18 cases of confirmed primary synovial sarcoma of the gastrointestinal tract. The neoplasms arose in 10 women and 8 men ranging in age from 23 to 81 years (mean: 50; median: 57.5 years). The tumors for which size was known ranged from 1.8 to 15.0 cm (mean: 5.2; median: 5.1 cm). Microscopically, 14 synovial sarcomas were of the monophasic type, 2 were biphasic, and 2 were poorly differentiated. Immunohistochemical analysis of 4 cases showed strong, diffuse staining for SS18::SSX (4/4 cases). Pancytokeratin and EMA immunohistochemistry were performed on 13 and 9 tumors, respectively, and each showed patchy-to-diffuse staining. By reverse-transcription PCR, 3 cases were positive for the SS18::SSX1, and 2 cases were positive for the SS18::SSX2 gene fusion. Six cases contained an SS18 gene rearrangement by fluorescence in situ hybridization, and next-generation sequencing identified an SS18::SSX2 gene fusion in one case. Clinical follow-up information was available for 9 patients (4 months to 4.6 years; mean, 2.8 y; median: 29 months), and one patient had a recent diagnosis. Three patients died of disease within 41 to 72 months (mean, 56 months) of their diagnosis. Five patients were alive without evidence of disease 4 to 52 months (mean, 17.6 months) after surgery; of whom 1 of the patients received additional chemotherapy treatment after surgery because of recurrence of the disease. A single patient was alive with intraabdominal recurrence 13 months after surgery. We conclude that synovial sarcoma of the gastrointestinal tract is an aggressive tumor, similar to its soft tissue counterpart, with adverse patient outcomes. It is important to distinguish it from morphologically similar gastrointestinal tract lesions that may have different treatment regimens and prognoses.
Topics: Male; Humans; Female; Young Adult; Adult; Middle Aged; Aged; Aged, 80 and over; Biomarkers, Tumor; Sarcoma, Synovial; In Situ Hybridization, Fluorescence; Proto-Oncogene Proteins; Oncogene Proteins, Fusion
PubMed: 37972927
DOI: 10.1016/j.modpat.2023.100383