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Journal For Immunotherapy of Cancer Jun 2021Synovial sarcoma (SS) is a rare cancer that disproportionately affects children and young adults. Cancer testis antigens (CTAs) are proteins that are expressed early in... (Review)
Review
Synovial sarcoma (SS) is a rare cancer that disproportionately affects children and young adults. Cancer testis antigens (CTAs) are proteins that are expressed early in embryonic development, but generally not expressed in normal tissue. They are aberrantly expressed in many different cancer types and are an attractive therapeutic target for immunotherapies. CTAs are expressed at high levels in SS. This high level of CTA expression makes SS an ideal cancer for treatment strategies aimed at harnessing the immune system to recognize aberrant CTA expression and fight against the cancer. Pivotal clinical trials are now underway, with the potential to dramatically alter the landscape of SS management and treatment from current standards of care. In this review, we describe the rationale for targeting CTAs in SS with a focus on NY-ESO-1 and MAGE-A4, the current state of vaccine and T-cell receptor-based therapies, and consider emerging opportunities for future development.
Topics: Antigens, Neoplasm; Cancer Vaccines; Child; Gene Expression Regulation, Neoplastic; Humans; Membrane Proteins; Neoplasm Proteins; Receptors, Antigen, T-Cell; Sarcoma, Synovial; T-Lymphocytes; Up-Regulation; Young Adult
PubMed: 34083416
DOI: 10.1136/jitc-2020-002072 -
Synovial Sarcoma of the Nerve-Clinical and Pathological Features: Case Series and Systematic Review.Neurosurgery Dec 2019Synovial sarcoma of the nerve is a rare entity with several cases and case series reported in the literature. Despite an improved understanding of the biology, the...
BACKGROUND
Synovial sarcoma of the nerve is a rare entity with several cases and case series reported in the literature. Despite an improved understanding of the biology, the clinical course is difficult to predict.
OBJECTIVE
To compile a series of patients with synovial sarcoma of the peripheral nerve (SSPN) and assess clinical and pathological factors and their contribution to survival and recurrence.
METHODS
Cases from 2 institutions collected in patients undergoing surgical intervention for SSPN. Systematic review including PubMed and Scopus databases were searched for related articles published from 1970 to December 2018. Eligibility criteria: (1) case reports or case series reporting on SSPN, (2) clinical course and/or pathological features of the tumor reported, and (3) articles published in English.
RESULTS
From patients treated at our institutions (13) the average follow-up period was 3.2 yr. Tumor recurrence was seen in 4 cases and death in 3. Systematic review of the literature yielded 44 additional cases with an average follow-up period of 3.6 yr. From pooled data, there were 10 recurrences and 7 deaths (20% and 14%, respectively). Adjuvant treatment used in 62.5% of cases. Immunohistochemical markers used in diagnosis varied widely; the most common are the following: Epithelial membrane antigen (EMA), cytokeratin, vimentin, cluster of differentiation (CD34), and transducin-like enhancer of split 1 (TLE1). Statistical analysis illustrated tumor size and use of chemotherapy to be negative predictors of survival. No other factors, clinically or from pathologist review, were correlated with recurrence or survival.
CONCLUSION
By combining cases from our institution with historical data and performing statistical analysis we show correlation between tumor size and death.
Topics: Biomarkers, Tumor; Humans; Peripheral Nervous System Neoplasms; Sarcoma, Synovial
PubMed: 31435657
DOI: 10.1093/neuros/nyz321 -
Journal of Clinical Oncology : Official... Dec 2021Synovial sarcoma (SS) is the second most common malignant soft tissue tumor in children. ARST0332 evaluated a risk-based treatment strategy for young patients with soft...
PURPOSE
Synovial sarcoma (SS) is the second most common malignant soft tissue tumor in children. ARST0332 evaluated a risk-based treatment strategy for young patients with soft tissue sarcoma designed to limit therapy for low-risk (LR) disease and to test neoadjuvant chemoradiotherapy for unresected higher-risk disease.
METHODS
Newly diagnosed patients with SS age < 30 years were assigned to four treatment arms based on disease features: A (surgery only), B (55.8 Gy radiotherapy [RT]), C (ifosfamide and doxorubicin [ID] chemotherapy plus 55.8 Gy RT), and D (neoadjuvant ID and 45 Gy RT, then surgery and RT boost based on margins followed by adjuvant ID). Patients treated in Arms A and B were considered LR, arms C and D without metastases as intermediate-risk (IR), and those with metastases as high-risk (HR).
RESULTS
Of the 146 patients with SS enrolled, 138 were eligible and evaluable: LR (46), IR (71), and HR (21). Tumors were 80% extremity, 70% > 5 cm, 70% high-grade, 62% invasive, 95% deep, and 15% metastatic. Treatment was on arm A (29.7%), B (3.6%), C (16.7%), and D (50%). There were no toxic deaths and four unexpected grade 4 adverse events. By risk group, at a median follow-up of 6.8 years, estimated 5-year event-free survival was LR 82%, IR 70%, and HR 8%, and overall survival was LR 98%, IR 89%, and HR 13%. After accounting for the features that defined risk category, none of the other patient or disease characteristics (age, sex, tumor site, tumor invasiveness, and depth) improved the risk stratification model.
CONCLUSION
The risk-based treatment strategy used in ARST0332 produced favorable outcomes in patients with nonmetastatic SS relative to historical controls despite using RT less frequently and at lower doses. The outcome for metastatic SS remains unsatisfactory and new therapies are urgently needed.
Topics: Adolescent; Adult; Child; Child, Preschool; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Infant; Male; Neoadjuvant Therapy; Prognosis; Prospective Studies; Sarcoma, Synovial; Survival Rate; Young Adult
PubMed: 34623899
DOI: 10.1200/JCO.21.01628 -
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 -
Cancer Medicine Jan 2023Synovial sarcoma (SS) accounts for 8%-10% of all soft-tissue sarcomas. Clinical presentation and outcomes vary, yet discrete risk groups based on validated prognostic...
BACKGROUND
Synovial sarcoma (SS) accounts for 8%-10% of all soft-tissue sarcomas. Clinical presentation and outcomes vary, yet discrete risk groups based on validated prognostic indices are not defined for the full spectrum of patients with SS.
METHODS
We performed a retrospective cohort study using data from the SEER (surveillance, epidemiology, and end results program) database of SS patients who were <70 years of age at diagnosis. We constructed a recursive partitioning model of overall survival using a training cohort of 1063 patients with variables: Age at diagnosis, sex, race, ethnicity, primary site, tumor size, tumor grade, and stage. Based on this model, we grouped patients into three risk groups and estimated 5-year overall survival for each group. We then applied these groups to a test cohort (n = 1063).
RESULTS
Our model identified three prognostic groups with significantly different overall survival: low risk (local/regional stage with either <21 years of age OR tumor <7.5 cm and female sex), intermediate-risk (local/regional stage, age ≥ 21 years with either male sex and tumor <7.5 cm OR any sex with appendicular anatomic location) and high risk (local/regional stage, age ≥ 21 years, tumor size ≥7.5 cm and non-appendicular location OR distant stage). Prognostic groups were applied to the test cohort, showing significantly different survival between groups (p < 0.0001).
CONCLUSIONS
Our analysis yields an intuitive risk-classification tree with discrete groups, which may provide useful information for researchers, patients, and clinicians. Prospective validation of this model may inform efforts at risk-stratifying treatment.
Topics: Humans; Young Adult; Adult; Sarcoma, Synovial; Retrospective Studies; Sarcoma; Prognosis; Risk Factors; SEER Program
PubMed: 35670308
DOI: 10.1002/cam4.4909 -
Swiss Medical Weekly Nov 2018Synovial sarcoma is a highly aggressive soft tissue malignancy that often affects adolescents and young adults. It is associated with a unique chromosomal translocation... (Review)
Review
Synovial sarcoma is a highly aggressive soft tissue malignancy that often affects adolescents and young adults. It is associated with a unique chromosomal translocation that results in the formation and expression of the fusion gene SS18-SSX, which underlies its pathogenesis. Although SS18-SSX provides a potentially unique therapeutic target, all attempts to neutralise it have been unsuccessful thus far. When complete surgical removal of the tumour fails, therapy is limited to largely ineffective cytotoxic drug regimens. Nevertheless, recent discoveries about the mechanisms of SS18-SSX protein function have provided insight into potential alternative therapeutic strategies. SS18-SSX displays oncogenic activity through protein-protein interactions and participation in chromatin remodelling complexes. This review summarises our current understanding of the function of SS18-SSX and the mechanisms by which it alters the epigenetic landscape of permissive cells to induce transformation and the subsequent development of synovial sarcoma.
Topics: Cell Transformation, Neoplastic; Chromatin; Epigenesis, Genetic; Humans; Neoplasm Proteins; Proto-Oncogene Proteins; Repressor Proteins; Sarcoma, Synovial; Translocation, Genetic
PubMed: 30506527
DOI: 10.4414/smw.2018.14667 -
Diagnostic Pathology Jul 2023Synovial sarcoma (SS) is a rare malignant soft tissue sarcoma that originates from primitive mesenchymal cells with epithelial differentiation potential. It is most... (Review)
Review
Synovial sarcoma (SS) is a rare malignant soft tissue sarcoma that originates from primitive mesenchymal cells with epithelial differentiation potential. It is most commonly found in the limbs and trunk. In the urinary system, it is mostly found in the kidneys. However, synovial sarcomas originating from the external urethra are extremely rare. Only one case of synovial sarcoma arising from the vulvar urethral orifice has been reported previously, and we report a second case of synovial sarcoma of the urethral orifice. In addition, a total of 16 vulvar synovial sarcomas were identified and the literature are analyzed in this report reviews from 1966 to the present.
Topics: Humans; Female; Sarcoma, Synovial; Urethra; Vulva; Sarcoma
PubMed: 37400856
DOI: 10.1186/s13000-023-01367-z -
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 -
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 -
BMC Cancer Feb 2019Synovial sarcoma is a relatively rare type of soft tissue sarcoma. The commonly observed symptom is a deep-seated palpable mass accompanied by pain or tenderness. Thus,... (Review)
Review
BACKGROUND
Synovial sarcoma is a relatively rare type of soft tissue sarcoma. The commonly observed symptom is a deep-seated palpable mass accompanied by pain or tenderness. Thus, it is considered a soft tissue sarcoma and rarely occurs primarily in bone. However, only few studies have been reported on intraosseous synovial sarcoma, and reports on cases with cytogenetic or molecular confirmation are even rarer. We report a case of intraosseous synovial sarcoma of the distal ulna that has been confirmed using histopathological examination and molecular analysis.
CASE PRESENTATION
A 77-year-old female was referred to our hospital with a 1-month history of right wrist pain after housework. Clinical and imaging findings suggested a benign bone tumor that was enhanced by Gd-DTPA. It was thought that the tumor was possibly an enchondroma. Initially, we planned to evaluate the benignancy of the tumor with intraoperative frozen section, followed by curettage and bone graft at one stage However, when considering carefully, characteristics of the tumor did not perfectly match those of any diagnostic categories including enchondroma. Therefore, an incisional biopsy was performed and revealed that the tumor was synovial sarcoma. Following an elaborate plan, the patient underwent a wide resection of the tumor at the distal part of the right ulna. Reverse transcription-polymerase chain reaction (RT-PCR) from the resected specimen and sequencing of RT-PCR products demonstrated a chimeric SYT-SSX1 transcript, confirming the diagnosis of synovial sarcoma.
CONCLUSIONS
Synovial sarcoma is seldom considered in differential diagnosis of bone tumors because it is difficult to line up such an unusual diagnosis as a differential diagnosis. When the lesion does not perfectly fit into any diagnostic category, when the initial image diagnosis appears unconvincing, biopsy and pathology are indicated, recalling Jaffe's triangle. According to these diagnostic processes, the patient successfully completed the treatment for this rare intraosseous synovial sarcoma, following a careful plan based on the preoperative diagnosis.
Topics: Aged; Biomarkers, Tumor; Bone Neoplasms; Diagnosis, Differential; Female; Humans; Oncogene Proteins, Fusion; Sarcoma, Synovial; Treatment Outcome; Ulna
PubMed: 30709383
DOI: 10.1186/s12885-019-5325-x