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Skeletal Radiology Apr 2024
Topics: Humans; Sarcoma, Synovial; Thigh; Lower Extremity; Leg; Chronic Pain; Soft Tissue Neoplasms
PubMed: 37819280
DOI: 10.1007/s00256-023-04475-5 -
Anticancer Research Sep 2018Synovial sarcoma is a soft tissue sarcoma that tends to affect young adults. There are few reports on primary synovial sarcoma of the thyroid and the aim of this study... (Review)
Review
BACKGROUND/AIM
Synovial sarcoma is a soft tissue sarcoma that tends to affect young adults. There are few reports on primary synovial sarcoma of the thyroid and the aim of this study was to document the clinical and pathological features of synovial sarcoma occurring at this site.
CASE PRESENTATION
A retrospective review of a prospectively maintained database was performed to identify patients with synovial sarcoma of the thyroid treated at the Royal Marsden Hospital between 2000 and 2017. Five patients were identified that underwent initial surgical management of localized disease. The mean age at presentation was 38 years, and male to female ratio was 4:1. In 3 out of 5 cases, the diagnosis of synovial sarcoma was made on the post-operative excision specimen. Two of these patients were treated at our institution on the development of metastatic disease. We conducted a literature review and identified 12 previously reported cases of synovial sarcoma of the thyroid.
CONCLUSION
Synovial sarcoma of the thyroid is rare and can be challenging to diagnose. The clinical presentation is typically indistinguishable from that of thyroid cancer and most cases are diagnosed post-operatively on an excision specimen. The clinical and pathological features are similar to synovial sarcoma arising at other sites. In our experience, the rarity of the diagnosis can lead to difficulty in establishing the correct diagnosis and determining the appropriate treatment pathway. It is important that physicians are aware of this diagnosis to facilitate prompt referral to a specialist centre, for specialist follow-up and treatment which is different to the thyroid cancer pathway.
Topics: Adolescent; Adult; Aged; Diagnosis, Differential; Disease Management; Female; Humans; Male; Middle Aged; Neoplasm Metastasis; Retrospective Studies; Sarcoma, Synovial; Thyroid Neoplasms
PubMed: 30194178
DOI: 10.21873/anticanres.12853 -
Pediatric Blood & Cancer Aug 2014Twenty-five to 32% of patients with synovial sarcoma (SS) relapse after appropriate treatment, and experience a poor outcome. Patients who can be salvaged by second-line... (Clinical Trial)
Clinical Trial
INTRODUCTION
Twenty-five to 32% of patients with synovial sarcoma (SS) relapse after appropriate treatment, and experience a poor outcome. Patients who can be salvaged by second-line therapy need to be more clearly identified.
PATIENTS AND METHODS
Data of patients treated in SFCE (Société Française des Cancers de l'Enfant) centers with an initial diagnosis of localized SS before the age of 18 years and treated from 1/1988 to 12/2008, and who experienced at least one relapse, were retrieved. After descriptive analysis, statistical analysis was performed to determine prognostic factors.
RESULTS
Thirty-seven patients were identified. First relapse occurred after a median interval of 24 months and was localized in 73.0% of cases and metastatic in 24.3% of cases. Treatment of relapse consisted of new surgery in 75.7% of cases, second-line chemotherapy in 73.0% of cases and radiotherapy in 48.6% of cases. Response rate to ifosfamide-based regimens was 36.4%. Overall, 70.3% patients achieved a second complete remission. Median 5-year-event-free survival was 32.8% and 5-year overall survival was 42.1%. Factors significantly correlated with better survival were primary tumor involving the limbs, age less than 12 years at diagnosis, absence of chemotherapy or radiotherapy as initial treatment and local relapse.
CONCLUSION
Despite its poor overall outcome, relapse of synovial sarcoma sometimes remains curable. Aggressive surgery, when possible, in combination with chemotherapy and radiotherapy is the recommended treatment. Ifosfamide-based regimens may remain effective in patients with relapsed SS. However, alternative therapies should be proposed in patients with poor prognostic factors.
Topics: Age Factors; Child; Child, Preschool; Disease-Free Survival; Female; Humans; Male; Neoplasm Recurrence, Local; Retrospective Studies; Sarcoma, Synovial; Survival Rate
PubMed: 24664883
DOI: 10.1002/pbc.25001 -
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 -
European Journal of Surgical Oncology :... Sep 2017The diagnosis of synovial sarcoma (SS) is currently based on clinical, morphological, immunohistochemical and cytogenetic data. Some of these factors such as grade and...
INTRODUCTION
The diagnosis of synovial sarcoma (SS) is currently based on clinical, morphological, immunohistochemical and cytogenetic data. Some of these factors such as grade and histology, specific translocations (SS18-SSX1 vs. SS18-SSX2) and the reduced expression of INI1, were proposed as prognostic variables. The aim of this study was to verify whether histological (grading and histology) and molecular (type of SSX translocation and INI1 expression) characteristics of SS influence the prognosis of the disease.
MATERIAL AND METHODS
We retrospectively evaluated 196 patients affected by SS of the extremities treated at our Institution (Istituto Ortopedico Rizzoli, Bologna, Italy). All cases were histologically revised and tumor grade was assessed according to the FNLCC system. Tissue specimens were retrospectively evaluated to check for SS18-SSX fusion type and INI1 expression.
RESULTS
Most SS were monophasic, 28% were biphasic. Eighty tumors (41%) were grade 3. Sixty percent harbored SSX1 translocation, 40% SSX2; 51% maintained the expression of INI1. Sarcoma specific survival (OS) was 56.6% at 5 years and 46.9% at 10 years. Prognosis was worse in those patients monophasic SS (p = 0.011) as in those with a grade 3 tumors (p = 0.083). No correlation was found neither between SSX fusion type nor INI1 expression and survival. LR-free survival was 78.9% at 5 years and 75.9% at 10 years. A higher LR rate was observed in tumors with SSX2 translocation and (p = 0.049) in grade 3 SS (0 = 0.028).
DISCUSSION
Our data confirm that not all cases of SS present the same severe outcome. High-risk patients identified on the basis of these parameters may qualify for an aggressive treatment approach.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Bone Neoplasms; Chemotherapy, Adjuvant; Child; Disease-Free Survival; Extremities; Female; Humans; Lung Neoplasms; Male; Middle Aged; Mutation; Neoplasm Grading; Neoplasm Proteins; Neoplasm Recurrence, Local; Oncogene Proteins, Fusion; Radiotherapy, Adjuvant; Reoperation; Repressor Proteins; Retrospective Studies; SMARCB1 Protein; Sarcoma, Synovial; Survival Rate; Translocation, Genetic; Young Adult
PubMed: 28579008
DOI: 10.1016/j.ejso.2017.05.020 -
Anticancer Research Sep 2023Synovial sarcoma (SS), a spindle cell sarcoma, typically occurs in the soft tissues of the extremities and rarely develops in the bones as a primary tumor. To our... (Review)
Review
BACKGROUND/AIM
Synovial sarcoma (SS), a spindle cell sarcoma, typically occurs in the soft tissues of the extremities and rarely develops in the bones as a primary tumor. To our knowledge, no case of SS in the metacarpal bone has been reported.
CASE REPORT
We report a case of primary SS of the metacarpal bone. Considering the clinical and imaging findings, SS was difficult to diagnose; therefore, we performed an open biopsy. Next, we performed a wide resection following the management guidelines for SS of the soft tissue. Immunohistochemistry (IHC) showed positive staining for SS18:SSX and SSX, and fluorescence in situ hybridization showed positive staining for the SS18 split. Owing to FNCLCC grade 3 tumor and the R1 margin, adjuvant chemotherapy with ifosfamide and doxorubicin was initiated. Finally, SS of the bone was diagnosed. Furthermore, we reviewed a total of 37 published cases of primary bone SS, including our case. Age and sex were almost the same in all cases of bone SS, and the most frequent site was the long bone in the lower extremity.
CONCLUSION
IHC for SS18::SSX and SSX antibodies are beneficial for diagnosing general SS and SS of the bone. Moreover, SS of the bone should be considered in the differential diagnosis of spindle cell sarcomas of the bone. Wide resection and chemotherapy are recommended as current treatment strategies, although further studies are required regarding treatment validity.
Topics: Humans; Sarcoma, Synovial; In Situ Hybridization, Fluorescence; Sarcoma; Osteosarcoma; Bone Neoplasms
PubMed: 37648290
DOI: 10.21873/anticanres.16617 -
The Journal of Histochemistry and... Jul 2023Synovial sarcoma is a rare malignant mesenchymal neoplasm mostly affecting young adults, characterized by a specific translocation which results in the fusion of the...
Synovial sarcoma is a rare malignant mesenchymal neoplasm mostly affecting young adults, characterized by a specific translocation which results in the fusion of the SS18 gene on chromosome 18 with one of the three highly homologous SSX genes on chromosome X. Its morphological diagnosis, especially in monophasic or poorly differentiated variants, can be challenging because histological features often overlap with other malignant mesenchymal tumors. Until recently, the differential diagnosis mostly relied on the use of cytogenetic or molecular analyses to detect the specific t(X;18)(p11;q11) translocation, thus virtually restricting its correct identification to referral centers with a high histological and molecular pathology workflow. The recently commercialized highly sensitive and fusion-specific SS18-SSX antibody has significantly improved the approach to these tumors, representing a relatively cheap and easy to access tool for synovial sarcoma diagnosis. Through a retrospective analysis of 79 synovial sarcomas and histological mimickers, this study confirms the usefulness of the SS18-SSX antibody in the diagnosis of synovial sarcoma, particularly focusing on its application in the pathological response evaluation after neoadjuvant treatment as well as its time- and cost-saving advantages. Finally, we here propose a new diagnostic algorithm to apply into the routine practice.
Topics: Young Adult; Humans; Repressor Proteins; Sarcoma, Synovial; Retrospective Studies; Oncogene Proteins, Fusion; Proto-Oncogene Proteins; Antibodies; Algorithms
PubMed: 37357741
DOI: 10.1369/00221554231184287 -
Journal of Surgical Oncology Sep 2022Synovial sarcoma is a rare malignant tumor that generally requires a multidisciplinary therapeutic approach. In this study we report the experience of a single surgeon,...
BACKGROUND
Synovial sarcoma is a rare malignant tumor that generally requires a multidisciplinary therapeutic approach. In this study we report the experience of a single surgeon, evaluating surgical and oncological outcomes of the cases he treated through his 30 years carrier.
METHODS
We enrolled patients treated surgically between 1988 and 2018. Surgical and medical treatments, as well as surgical and oncological results, were investigated.
RESULTS
One hundred and thirty cases were included. Surgical resection was carried out achieving wide margins in 90% of the cases. At their latest follow-up, 76 patients were continuously disease free, 16 were no evidence of disease, and other 16 were alive with disease. Twenty cases were dead of disease and two dead of other causes. Twenty-five patients (19%) had local recurrence of synovial sarcoma through their postoperative intercourse. Thirty-seven patients (28%) were diagnosed with at least a metastasis during their follow-up. The global survival of our population, at each patient's latest follow-up, was 82%. Cases with tumor size above 5 cm had a significantly higher risk to develop metastasis (p = 0.002).
CONCLUSIONS
Synovial sarcoma is a threatening disease and represents a challenge for oncological physicians and surgeons. Early diagnosis and multidisciplinary approach are mandatory to limit the spread of synovial sarcomas, maximizing the effectiveness of surgery and the other treatments.
Topics: Disease-Free Survival; Humans; Male; Margins of Excision; Neoplasm Recurrence, Local; Retrospective Studies; Sarcoma, Synovial; Surgeons
PubMed: 35670050
DOI: 10.1002/jso.26976 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Apr 2017Synovial sarcoma of the nasal cavity and sinus are rare,comprise less than 0.1% of all soft tissue malignancies. Synovial sarcoma is a malignant mesenchymal tumor with... (Review)
Review
Synovial sarcoma of the nasal cavity and sinus are rare,comprise less than 0.1% of all soft tissue malignancies. Synovial sarcoma is a malignant mesenchymal tumor with variable epithelial differentiation, which is defined by the presence of a specific chromosomal translocation that generates SS18-SSX fusion oncogenes. The treatment include surgery,radiotherapy,chemotherapy. As the delicate anatomy of the nasal cavity and sinus limits the ability to obtain wide surgical margins. This maybe the reason why there is a higher local recurrence rate and worse disease-specific survival in head and neck sarcomas compared to other sites.
Topics: Humans; Nasal Cavity; Nose Neoplasms; Oncogene Proteins, Fusion; Paranasal Sinuses; Sarcoma, Synovial
PubMed: 29871316
DOI: 10.13201/j.issn.1001-1781.2017.07.022 -
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