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Journal of Neurosurgery. Spine Jun 2024Synovial sarcoma (SS) is a relatively rare type of soft-tissue sarcoma that is commonly treated with surgery, radiation, chemotherapy, and palliative care. Stereotactic... (Review)
Review
OBJECTIVE
Synovial sarcoma (SS) is a relatively rare type of soft-tissue sarcoma that is commonly treated with surgery, radiation, chemotherapy, and palliative care. Stereotactic radiosurgery (SRS) is an emerging approach that shows promise in treating CNS conditions, but it has not been studied for SS. The authors present a systematic review that explores the effectiveness of different treatments, with a focus on SRS, for managing spinal SS.
METHODS
A systematic PubMed search was conducted that covered studies from 1964 to 2022, yielding 70 relevant studies. Inclusion criteria encompassed primary and metastatic spinal SS, various treatment modalities, patient age 17 years or older, English-language studies, retrospective series, and case reports. Based on these criteria, 26 studies were included in this review and 44 were excluded.
RESULTS
Of the included studies, 15 patients from 9 studies were treated with surgical intervention followed by both conventional radiotherapy (RT) and chemotherapy, 10 patients from 10 studies were treated with surgery followed by RT, 5 studies comprising 8 patients were exclusively treated with surgery, 5 cases in 3 studies were treated with surgery plus concomitant chemotherapy, 4 patients in 2 studies were treated with SRS, and only 1 study reported treatment without surgery and with chemotherapy and RT. The median progression-free survival and overall survival periods observed in the SRS-treated patients were 37 months and 60 months, respectively, which were higher than those of any other treatment method or combination used.
CONCLUSIONS
The authors' study offers a thorough review of spinal SS treatments. They are hopeful that this will aid clinicians in informed decision-making for better patient outcomes.
Topics: Humans; Sarcoma, Synovial; Spinal Neoplasms; Radiosurgery; Combined Modality Therapy
PubMed: 38489819
DOI: 10.3171/2024.1.SPINE231184 -
Polish Journal of Pathology : Official... 2020Synovial sarcoma is a rare mesenchymal malignant neoplasm that presents a specific t(X;18) translocation forming SS18(SYT)-SSX chimera gene. It is most commonly seen in...
Synovial sarcoma is a rare mesenchymal malignant neoplasm that presents a specific t(X;18) translocation forming SS18(SYT)-SSX chimera gene. It is most commonly seen in soft tissues of the extremities. The digestive tract is an exceptional site of involvement. We report a case of primary gastric synovial sarcoma in a 48-year-old female. Differential diagnosis of synovial sarcoma from other spindle cell, mesenchymal and cytokeratin-positive tumors is critical for the treatment and prognosis. Immunohistochemistry studies and molecular analysis are required to settle a proper diagnosis.
Topics: Female; Humans; Middle Aged; Immunohistochemistry; Oncogene Proteins, Fusion; Sarcoma, Synovial; Translocation, Genetic
PubMed: 32729309
DOI: 10.5114/pjp.2020.97024 -
Cancers Oct 2022The aim of this study was to systematically review the literature of sarcoma of the parotid gland in order to analyze the main factors affecting survival rate. A... (Review)
Review
Systematic Review of Parotid Gland Sarcomas: Multi-Variate Analysis of Clinicopathologic Findings, Therapeutic Approaches and Oncological Outcomes That Affect Survival Rate.
The aim of this study was to systematically review the literature of sarcoma of the parotid gland in order to analyze the main factors affecting survival rate. A systematic literature review was performed between January 1990 to November 2021, and 88 patients affected by parotid gland sarcomas were included. The most common histological types were Rhabdomyosarcoma and Synovial Sarcoma. From our review, it emerges that primary sarcomas of the parotid glands are locally aggressive but show low tendency to metastasize to the lymph nodes of the neck and that surgery (i.e., total or radical parotidectomy) is the main approach for their treatment. The global overall survival (OS) is 52% at 5 years and 34.1% at 10 years. The OS for T1, T2, T3, T4 tumor at 5 years of follow up is 80.0%, 66.5%, 56.7% and 33.3%, respectively. Size/extension at the diagnosis and the sarcoma's histotype are the most important prognostic factors. Multivariate analysis showed that surgery (total or radical parotidectomy) performed on the tumor ( = 0.0008) was the only parameter that significantly affected the OS. Among the other variables, age (younger), use of adjuvant therapy and lymph node metastasis showed borderline significative values ( = 0.05). Our analysis suggests that, when a primitive parotid sarcoma is diagnosed, total or radical parotidectomy should be performed at any age independent of tumor histology. Because regional lymph node metastases from parotid sarcomas are uncommon, alternative strategies (e.g., close follow-up by imaging and evaluation of sentinel lymph nodes) should be pursued before lymph node (selective/radical) dissection.
PubMed: 36230786
DOI: 10.3390/cancers14194862 -
Pediatric Reports Jan 2022In cases with solid tumors, preoperative radiological investigations provide valuable information on the anatomy of the tumor and the adjoining structures, thus helping... (Review)
Review
BACKGROUND
In cases with solid tumors, preoperative radiological investigations provide valuable information on the anatomy of the tumor and the adjoining structures, thus helping in operative planning. However, due to a two-dimensional view in these investigations, a detailed spatial relationship is difficult to decipher. In contrast, three-dimensional (3D) printing technology provides a precise topographic view to perform safe surgical resections of these tumors. This systematic review aimed to summarize and analyze current evidence on the utility of 3D printing in pediatric extra-cranial solid tumors.
METHODS
The present study was registered on PROSPERO-international prospective register of systematic reviews (registration number: CRD42020206022). PubMed, Embase, SCOPUS, and Google Scholar databases were explored with appropriate search criteria to select the relevant studies. Data were extracted to study the bibliographic information of each article, the number of patients in each study, age of the patient(s), type of tumor, organ of involvement, application of 3D printing (surgical planning, training, and/or parental education). The details of 3D printing, such as type of imaging used, software details, printing technique, printing material, and cost were also synthesized.
RESULTS
Eight studies were finally included in the systematic review. Three-dimensional printing technology was used in thirty children with Wilms tumor (n = 13), neuroblastoma (n = 7), hepatic tumors (n = 8), retroperitoneal tumor (n = 1), and synovial sarcoma (n = 1). Among the included studies, the technology was utilized for preoperative surgical planning (five studies), improved understanding of the surgical anatomy of solid organs (two studies), and improving the parental understanding of the tumor and its management (one study). Computed tomography and magnetic resonance imaging were either performed alone or in combination for radiological evaluation in these children. Different types of printers and printing materials were used in the included studies. The cost of the 3D printed models and time involved (range 10 h to 4-5 days) were reported by two studies each.
CONCLUSIONS
3D printed models can be of great assistance to pediatric surgeons in understanding the spatial relationships of tumors with the adjacent anatomic structures. They also facilitate the understanding of families, improving doctor-patient communication.
PubMed: 35076594
DOI: 10.3390/pediatric14010006 -
Future Oncology (London, England) Dec 2020Synovial sarcoma (SS) is a rare, aggressive soft tissue sarcoma with a poor prognosis after metastasis. The objective of this study was to conduct a systematic review...
Synovial sarcoma (SS) is a rare, aggressive soft tissue sarcoma with a poor prognosis after metastasis. The objective of this study was to conduct a systematic review of the clinical evidence for therapeutic options for adults with metastatic or advanced SS. Relevant databases were searched with predefined keywords. Thirty-nine publications reported clinical data for systemic treatment and other interventions. Data on survival outcomes varied but were generally poor (progression-free survival: 1.0-7.7 months; overall survival: 6.7-29.2 months) for adults with metastatic and advanced SS. A high frequency of neutropenia with systemic treatment and low quality of life post-progression were reported. Reported evidence suggests poor outcomes in adults with metastatic and advanced SS and the need for the development of new treatment modalities.
Topics: Adult; Humans; Neoplasm Metastasis; Quality of Life; Sarcoma, Synovial
PubMed: 32851862
DOI: 10.2217/fon-2020-0575 -
Cancers Feb 2021Surgery is the mainstay of treatment for localized soft tissue sarcomas (STS). The curative treatment highly depends on complete tumor resection, as positive margins are... (Review)
Review
Surgery is the mainstay of treatment for localized soft tissue sarcomas (STS). The curative treatment highly depends on complete tumor resection, as positive margins are associated with local recurrence (LR) and prognosis. However, determining the tumor margin during surgery is challenging. Real-time tumor-specific imaging can facilitate complete resection by visualizing tumor tissue during surgery. Unfortunately, STS specific tracers are presently not clinically available. In this review, STS-associated cell surface-expressed biomarkers, which are currently already clinically targeted with monoclonal antibodies for therapeutic purposes, are evaluated for their use in near-infrared fluorescence (NIRF) imaging of STS. Clinically targeted biomarkers in STS were extracted from clinical trial registers and a PubMed search was performed. Data on biomarker characteristics, sample size, percentage of biomarker-positive STS samples, pattern of biomarker expression, biomarker internalization features, and previous applications of the biomarker in imaging were extracted. The biomarkers were ranked utilizing a previously described scoring system. Eleven cell surface-expressed biomarkers were identified from which 7 were selected as potential biomarkers for NIRF imaging: TEM1, VEGFR-1, EGFR, VEGFR-2, IGF-1R, PDGFRα, and CD40. Promising biomarkers in common and aggressive STS subtypes are TEM1 for myxofibrosarcoma, TEM1, and PDGFRα for undifferentiated soft tissue sarcoma and EGFR for synovial sarcoma.
PubMed: 33535618
DOI: 10.3390/cancers13030557 -
Journal of Thoracic Disease Apr 2021Soft tissue sarcoma (STS) tend to metastasis to the lungs. Pulmonary metastasectomy seems to be a common practice always when plausible. The objective of this article...
BACKGROUND
Soft tissue sarcoma (STS) tend to metastasis to the lungs. Pulmonary metastasectomy seems to be a common practice always when plausible. The objective of this article was to review systematically the results of a literature search on pulmonary metastasectomy for STSs published in the last ten years and to offer a brief overview about the current practice as well.
METHODS
Eight retrospective studies published in the period 2010-2020, which included patients with pulmonary metastases and metastasectomy were selected. Indication for surgery, survival rate and factors influencing survival were the primary outcomes, while further interesting findings in the studies were also collected and evaluated.
RESULTS
Cumulative 1,004 patients participated in these studies. The most common histological types were leiomyosarcoma, malignant fibrous histiocytoma (MFH) and synovial sarcoma, being present together at 60% of the study population. Five-year survival was reported to be in the range from 20-58%, better survival going along with a fewer (preferably one) metastases, longer disease free interval (DFI) and R0 resection in most of the cases.
CONCLUSIONS
Complete resection of the metastatic lesions seems to be the most effective treatment for long-term survival, or even achieving cure in selected patients. At selection of the patients amenable for surgery, a high probability of R0 resection, as well as a disease free period of at least 12 months should perhaps bear a higher specific value.
PubMed: 34012614
DOI: 10.21037/jtd-2019-pm-13 -
Cancer Treatment Reviews Apr 2020To make recommendations on the indications for molecular testing regarding the diagnosis, prediction of prognosis, and treatment selection in adult patients with s oft... (Meta-Analysis)
Meta-Analysis
AIMS
To make recommendations on the indications for molecular testing regarding the diagnosis, prediction of prognosis, and treatment selection in adult patients with s oft tissue sarcomas (STS) excluding gastrointestinal stromal tumour.
MATERIALS AND METHODS
This guideline was developed by the Cancer Care Ontario's Program in Evidence-Based Care (PEBC) and the Sarcoma Disease Site Group (DSG). The medline, embase, and Cochrane Library databases, main guideline websites, abstracts of relevant annual meetings, and PROSPERO databases were searched (January 2005 to October 2016). Internal and external reviews were conducted, with final approval by the PEBC and the Sarcoma DSG.
RESULTS
Based on the available evidence, we made three S trong Recommendations, 14 Recommendations, 9 Qualified Statements, and seven No Recommendations. The three Strong Recommendations include: i) MDM2 amplification by fluorescence in situ hybridization (FISH) is recommended as a sensitive and specific test to differentiate patients with atypical lipomatous tumour/well-differentiated liposarcoma, or dedifferentiated liposarcoma from lipoma or other STS in the differential diagnosis; ii) SS18 (SYT) break-apart by FISH or SS18-SSX (SYT-SSX) fusion by reverse transcription-polymerase chain reaction is recommended as a sensitive and specific test to differentiate patients with synovial sarcoma from other sarcomas; iii) CTNNB1 S45F mutation by polymerase chain reaction is recommended as a prognostic factor for poor recurrence-free survival in patients with desmoid tumours.
CONCLUSION
This guideline may serve as a framework for the thoughtful implementation of molecular studies at cancer centres and other jurisdictions. Some of the recommendations may need to be updated when new evidence appears in the future.
Topics: Biomarkers, Tumor; Evidence-Based Medicine; Female; Gastrointestinal Stromal Tumors; Genetic Testing; Humans; Male; Oncogene Proteins, Fusion; Ontario; Practice Guidelines as Topic; Prognosis; Sarcoma; Sensitivity and Specificity; Soft Tissue Neoplasms
PubMed: 32092619
DOI: 10.1016/j.ctrv.2020.101987