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Current Oncology (Toronto, Ont.) May 2021Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5-10% of all STS. Synovial sarcoma differs from other STS by the... (Review)
Review
Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5-10% of all STS. Synovial sarcoma differs from other STS by the relatively young age at diagnosis and clinical presentation. Synovial sarcomas have unique genomic characteristics and are driven by a pathognomonic t(X;18) chromosomal translocation and subsequent formation of the SS18:SSX fusion oncogenes. Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. Increasing size, age and tumor grade have been demonstrated to be negative predictive factors for both local disease recurrence and metastasis. Wide surgical excision remains the standard of care for definitive treatment with adjuvant radiation utilized for larger and deeper lesions. There remains controversy surrounding the role of chemotherapy in the treatment of SS and there appears to be survival benefit in certain populations. As the understanding of the molecular and immunologic characteristics of SS evolve, several potential systematic therapies have been proposed.
Topics: Humans; Neoplasm Recurrence, Local; Oncogene Proteins, Fusion; Sarcoma, Synovial; Soft Tissue Neoplasms; Translocation, Genetic
PubMed: 34069748
DOI: 10.3390/curroncol28030177 -
Current Treatment Options in Oncology Mar 2023Synovial sarcoma (SS) is a fusion-driven subtype of sarcoma that is a more chemo-sensitive subtype of soft tissue sarcoma. While chemotherapy options are currently... (Review)
Review
Synovial sarcoma (SS) is a fusion-driven subtype of sarcoma that is a more chemo-sensitive subtype of soft tissue sarcoma. While chemotherapy options are currently standard of care, our fundamental understanding of the biology of SS is driving new therapies. We will review the current standard of care, as well as the current therapies showing promise in a clinical trial. It is our hope that by encouraging participation in clinical trials, the fundamental therapies available for SS will change the current treatment paradigm.
Topics: Humans; Sarcoma, Synovial; Sarcoma; Soft Tissue Neoplasms
PubMed: 36867389
DOI: 10.1007/s11864-023-01056-5 -
Annals of Diagnostic Pathology Dec 1998Synovial sarcoma is a rare soft tissue tumor of children and adults that is unrelated to synovium and can occur in almost any part of the body. The familiar biphasic... (Review)
Review
Synovial sarcoma is a rare soft tissue tumor of children and adults that is unrelated to synovium and can occur in almost any part of the body. The familiar biphasic synovial sarcoma has discernible glandular or solid epithelial structures, and monophasic forms have characteristic ovoid or spindle cells with only immunohistochemical or ultrastructural evidence of epithelial differentiation. There are several morphologic patterns, including myxoid and hemangiopericytic, and behaviorally distinct calcifying, ossifying, and poorly differentiated subtypes can be recognized. Most synovial sarcomas are immunoreactive for cytokeratin, epithelial membrane antigen, and bc12 protein, and negative for CD34, and many express S100 protein and CD99 (MIC2). Nearly all synovial sarcomas have a specific t(x;18) (p11.2;q11.2) chromosomal abnormality, resulting in fusion of either of two variants of the SSX gene with the SYT gene; the genetic features may relate to morphology and outcome. The differential diagnosis can include a wide range of spindled, polygonal, or round cell sarcomas. Clinically, there have been marked recent improvements in local control of disease and lesser ones in management of metastases. The pathology, differential diagnosis, and behavior of this unique tumor are reviewed.
Topics: Adult; Child; Demography; Diagnosis, Differential; History, 19th Century; History, 20th Century; Humans; Prognosis; Sarcoma, Synovial; Soft Tissue Neoplasms; Terminology as Topic
PubMed: 9930576
DOI: 10.1016/s1092-9134(98)80042-7 -
Medicina Clinica Feb 2022
Topics: Humans; Sarcoma, Synovial; Translocation, Genetic
PubMed: 34666901
DOI: 10.1016/j.medcli.2021.07.019 -
Journal of Basic and Clinical... Mar 2023Sarcoma is defined as a tumor located in the thoracic cavity. However, sarcoma can occur on every side of the body. Synovial sarcoma is a rare soft tissue tumor... (Review)
Review
Sarcoma is defined as a tumor located in the thoracic cavity. However, sarcoma can occur on every side of the body. Synovial sarcoma is a rare soft tissue tumor originating from pluripotent with a high malignancy rate. The most common predilection of synovial sarcoma is in the joints. Primary synovial sarcoma of the lung and mediastinum are rare tumors and generally malignant. There are only a few cases have been reported. Definite diagnosis is made by histopathological, immunohistochemistry, and cytogenetic examination. The management strategy for synovial sarcoma requires multimodality treatment with surgery, chemotherapy, and radiotherapy. However, effective and relatively non-toxic therapy for primary synovial sarcoma is still developed. The five years life expectancy is higher if the patient received adjuvant radiotherapy and/or chemotherapy after surgery.
Topics: Humans; Sarcoma, Synovial; Mediastinal Neoplasms; Mediastinum; Lung; Lung Neoplasms
PubMed: 36800987
DOI: 10.1515/jbcpp-2022-0286 -
Journal of Medical Case Reports Jan 2021Synovial sarcomas are infrequent malignant tumors occurring mostly in adolescents and young adults. The occurrence of synovial sarcoma in the hand-wrist area is rare (4... (Review)
Review
BACKGROUND
Synovial sarcomas are infrequent malignant tumors occurring mostly in adolescents and young adults. The occurrence of synovial sarcoma in the hand-wrist area is rare (4 to 8.5% of all synovial sarcomas in different studies).
CASE PRESENTATION
This report documents an uncommon case of synovial sarcoma occurring in the hand-wrist of a 69-year-old Caucasian woman. She was subsequently treated with surgical excision and radiotherapy without recurrence after follow up.
CONCLUSIONS
This paper aims to characterize the demographic, pathologic, and clinical features with a literature review. The present literature review confirms that hand-wrist synovial sarcomas are more frequent among males and subjects 10 to 40 years old. Most cases in this location are usually not larger than 5 cm in size. The five-year survival rate is higher than that reported in a previous review on hand synovial sarcomas, and this suggests an improved survival in recent decades.
Topics: Aged; Diagnostic Errors; Female; Ganglion Cysts; Hand; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Proto-Oncogene Proteins; Radiotherapy, Adjuvant; Reoperation; Repressor Proteins; Sarcoma, Synovial; Soft Tissue Neoplasms; Wrist
PubMed: 33453728
DOI: 10.1186/s13256-020-02613-4 -
ANZ Journal of Surgery Apr 2021
Topics: Esophagus; Humans; Sarcoma, Synovial
PubMed: 32857866
DOI: 10.1111/ans.16237 -
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 -
Journal of Stomatology, Oral and... Nov 2021Synovial sarcoma is a high-grade malignancy that seldom manifests in the head and neck. The purpose of this paper is to provide a comprehensive review of the existing... (Review)
Review
Synovial sarcoma is a high-grade malignancy that seldom manifests in the head and neck. The purpose of this paper is to provide a comprehensive review of the existing literature on primary HNSS. This review study includes case reports, case series and retrospective cohort studies on primary HNSS published in the English language. All cases identified via PubMed were analyzed individually. Articles were included according to specified eligibility criteria. The total number of cases analyzed was 243 (91 case reports, 53 cases from 16 case series, and 99 cases from 8 retrospective cohort studies). The average age at the time of HNSS diagnosis was 33.5 years. The ratio of male-to-female was 1.64:1. The most frequently occurring histologic subtype was biphasic (59.2%). The most common location was the neck. Hoarseness was the most common presenting symptom. Almost all patients (98.3%) underwent surgical excision. 55.6% of patients received adjuvant radiotherapy. A smaller number of patients (30.7%) received adjuvant chemotherapy. 60.3% of patients were alive with no evidence of disease at follow-up while 28.0% of patients died of HNSS at follow-up. The average follow-up period was 52.0 months. Synovial sarcoma is a rare head and neck malignancy. Surgical excision with adjuvant radiation is the most frequently employed treatment modality. Regardless of treatment modality, the prognosis of the tumor is guarded.
Topics: Female; Head and Neck Neoplasms; Humans; Male; Prognosis; Radiotherapy, Adjuvant; Retrospective Studies; Sarcoma, Synovial
PubMed: 33301946
DOI: 10.1016/j.jormas.2020.12.001 -
The American Journal of Surgical... 1986The contributions of Dr. Arthur Purdy Stout to surgical pathology and his observations pertaining to soft tissue tumors (and, more specifically, to synovial sarcoma) are... (Review)
Review
The contributions of Dr. Arthur Purdy Stout to surgical pathology and his observations pertaining to soft tissue tumors (and, more specifically, to synovial sarcoma) are briefly reviewed. In addition, a report on 185 patients treated at the Mayo Clinic for synovial sarcoma is reviewed. In that study, histologic subclassification stratified the tumors as follows: 33% with a predominant biphasic pattern, 31% with a monophasic pattern, and 36% with a mixed pattern. For all 185 patients, the 5-year survival rate was 38% and the 10-year rate was 23%. For patients treated since 1960, the survival rates were 55% at 5 years and 38% at 10 years. Female patients, young patients, and patients with tumors less than 5 cm in diameter had significantly higher survival rates than did their counterparts. Although histologic subtyping did not reveal significant differences in patient survival, patients with glandular differentiation of the epithelial elements seemed to do better.
Topics: Cell Transformation, Neoplastic; Humans; Lymphatic Metastasis; Prognosis; Sarcoma, Synovial
PubMed: 3035951
DOI: No ID Found