-
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 -
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 -
European Journal of Surgical Oncology :... Sep 2022Synovial sarcoma (SS) is a malignancy with high metastatic potential. The role of metastasectomy in SS is unclear, with limited data on prognostic factors and clinical... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Synovial sarcoma (SS) is a malignancy with high metastatic potential. The role of metastasectomy in SS is unclear, with limited data on prognostic factors and clinical outcomes. In this systematic review, we evaluate the survival outcomes post-metastasectomy for patients with SS.
METHODS
A systematic review was undertaken following PRISMA guidelines. English studies reporting survival outcomes among adults and children with SS undergoing metastasectomy were evaluated. Databases were searched from inception to May 31, 2021, and included Medline, Embase, Cochrane Central Register of Controlled Trials, and ClinicalTrials.gov. Two reviewers independently undertook literature evaluation and screening, data extraction and grading of studies. Risk of bias assessments utilized the Newcastle-Ottawa Quality Assessment Scale for Cohort Studies and the Joanna Briggs Institute Critical Appraisal Checklist for Case Series. Qualitative data was summarized in descriptive format, and survival outcome data were assessed for meta-analysis.
RESULTS
Thirteen retrospective studies, published between 1993 and 2017, were included, four were cohort studies, and nine were case series. A total of 598 patients with SS were included, of whom 462 had metastatic pulmonary disease, and 309 underwent metastasectomy. The median ages of the study cohorts ranged from 14 to 51 years. The median survival period after metastasectomy ranged from 21 to 80 months. Patients who underwent metastasectomy had a lower risk of mortality compared to those who did not (pooled HR 0.26 95% CI 0.14-0.49). The most common prognostic factors associated with survival included a disease-free interval of greater than 12 months and complete resection of the metastases.
DISCUSSION
Although the level of evidence is low, retrospective studies support a clinical advantage for metastasectomy in selected patients with metastatic SS.
FUNDING
This was not a funded study.
REGISTRATION
This protocol has been registered within the international prospective register of systematic reviews (PROSPERO) database (registration ID: CRD42019126906).
Topics: Adolescent; Adult; Child; Disease-Free Survival; Humans; Metastasectomy; Middle Aged; Retrospective Studies; Sarcoma, Synovial; Young Adult
PubMed: 35672231
DOI: 10.1016/j.ejso.2022.05.022 -
Clinical Neurology and Neurosurgery Mar 2022Primary central nervous system (CNS) sarcomas represent a heterogeneous group of rare neoplasms with unclear etiology. Available data on clinical characteristics,... (Review)
Review
BACKGROUND
Primary central nervous system (CNS) sarcomas represent a heterogeneous group of rare neoplasms with unclear etiology. Available data on clinical characteristics, treatment strategies, and survival are scarce. We comprehensively reviewed management strategies and outcomes of primary CNS sarcomas in adults.
METHODS
PubMed, Scopus, and Cochrane were search following the PRISMA guidelines to include studies on primary CNS sarcomas in adults. Clinical features, management strategies, and survival were analyzed.
RESULTS
We included 9 studies comprising 78 patients. Primary CNS sarcomas were mostly intracranial (87.2%), frequently located in the parietal (17.9%), frontal (14.1%), and temporal (14.1%) lobes. Spinal CNS sarcomas were found in 10 patients (12.8%). The most common tumor histology were fibrosarcoma (16.7%), intracranial synovial sarcoma (12.8%), extraosseous mesenchymal chondrosarcoma (11.5%), perivascular sarcoma (11.5%), reticulum cell sarcoma (11.5%), and myeloid sarcoma (9%). Partial resection (57.7%) was preferred over complete resection (42.3%), and 43 patients (55.1%) received adjuvant treatments: radiotherapy (51.3%) and/or systemic chemotherapy (20.5%). 21 patients experienced CNS sarcomas recurrences, with a median progression-free survival of 9 months (range, 4-48). At last follow-up, 60 patients (76.9%) were dead, with a median overall survival of 9 months (0.1-396). Overall survival was significantly longer in patients with fibrosarcoma (p = 0.001).
CONCLUSION
Surgical resection coupled with adjuvant chemotherapy or radiation has historically been the cornerstone treatment for CNS sarcoma but showed poor local control and dismal survival. A better understanding of the CNS sarcoma microenvironment may favor the development of tailored strategies aimed at improving survival.
Topics: Adult; Central Nervous System; Central Nervous System Neoplasms; Chemotherapy, Adjuvant; Fibrosarcoma; Humans; Retrospective Studies; Sarcoma; Tumor Microenvironment
PubMed: 35151057
DOI: 10.1016/j.clineuro.2022.107127 -
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 -
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 -
Clinical Journal of Gastroenterology Aug 2021Worldwide, 5-10% of soft tissue sarcoma cases in adults have been attributed to synovial sarcoma. It is often reported to occur near the joints of the arm, neck, and leg...
Worldwide, 5-10% of soft tissue sarcoma cases in adults have been attributed to synovial sarcoma. It is often reported to occur near the joints of the arm, neck, and leg but rarely in the gastrointestinal tract. In this study, we report a case of synovial sarcoma arising in the stomach of a 59-year-old woman. Gastrointestinal endoscopy revealed an ulcerative and hemorrhagic tumor with marginal elevation in the fundus. Histological study showed that the tumor was composed of tightly packed spindle cells in bundles, and one of its component demonstrated significant mitotic activity (> 40/10 high-power fields) in several areas. The diagnosis was confirmed by the evidence of SS18 gene rearrangement, according to immunohistochemistry study, (including a novel SS18-SSX fusion-specific antibody), fluorescent in situ hybridization, and the identification of the SS18-SSX1 and SS18-SSX1/2/4 fusion transcripts using reverse-transcript polymerase chain reaction. No evidence of local recurrence or distant metastasis has been found in the more than 5 years since. Distinguishing synovial sarcoma in the digestive tract from other mesenchymal neoplasms, such as gastrointestinal stromal tumor, may be difficult, especially when spindle-shaped cell proliferation is predominant, as in our patient. Therefore, morphological, immunohistological, and molecular evaluations are important for a comprehensive diagnosis.
Topics: Adult; Biomarkers, Tumor; Female; Humans; In Situ Hybridization, Fluorescence; Middle Aged; Neoplasm Recurrence, Local; Oncogene Proteins, Fusion; Sarcoma, Synovial; Stomach
PubMed: 33844129
DOI: 10.1007/s12328-021-01408-4 -
Japanese Journal of Clinical Oncology May 2021Synovial sarcoma is the most common soft tissue sarcomas among childhood and adolescents, accounting for 8-10% of all soft tissue sarcoma. Synovial sarcoma is considered...
OBJECTIVE
Synovial sarcoma is the most common soft tissue sarcomas among childhood and adolescents, accounting for 8-10% of all soft tissue sarcoma. Synovial sarcoma is considered a relatively chemosensitive tumor compared with other soft tissue sarcomas. However, the role of perioperative chemotherapy in synovial sarcoma remains controversial. The purpose of this systematic review is to evaluate the role of perioperative chemotherapy in childhood and adolescent patients with synovial sarcoma.
METHODS
We evaluated studies published between 1 January 1990 and 31 December 2017. The following databases were searched: MEDLINE, Cochrane database (via PubMed) and Ichushi (in Japanese).
RESULTS
The search yielded 216 articles in English and Japanese. After the initial screening, based on the title and abstract, 160 articles were excluded. As a second screening, we then assessed the full text of the remaining 56 articles for eligibility. Finally, 10 articles were included in the systematic review. Surgical resection with R0 margin alone was recommended because of the excellent results of two prospective studies. Meta-analysis was performed using data from two retrospective studies of 261 patients. Perioperative chemotherapy did not have a significant effect on survival and event-free survival.
CONCLUSIONS
We weakly do not recommend perioperative chemotherapy in patients with non-metastatic synovial sarcoma ≤ 5 cm when R0 resection is acquired. There was no consensus concerning the role of perioperative chemotherapy in patients with synovial sarcoma > 5 cm or those with ≤5 cm who undergo R1 or R2 resection.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Male; Prospective Studies; Retrospective Studies; Sarcoma; Sarcoma, Synovial; Soft Tissue Neoplasms
PubMed: 33822979
DOI: 10.1093/jjco/hyab039 -
Current Urology Reports Mar 2021To update epidemiological, diagnostic, and therapeutic information on primary synovial sarcoma of the kidney.
PURPOSE OF REVIEW
To update epidemiological, diagnostic, and therapeutic information on primary synovial sarcoma of the kidney.
RECENT FINDINGS
A total of 96 studies were analyzed; age at presentation was 38.6±14.2 years, predominant location of tumor was right kidney; frequent reported symptoms at diagnosis were hematuria and pain. For definitive diagnosis, cytogenetic technique was used. Detected oncogene was available in 37.8% cases with fusion of SS18-SSX in most patients. Surgery is treatment of choice, with adjuvant chemotherapy; most frequently ifosfamide-based associated with doxorubicin or epirubicin. Overall median survival was 34 months. Mortality was 29% of the cases which reported death and the recurrence rate was 39.8%. Risk of death was increased in patients with metastases at diagnosis Primary RSS occurs more often in young men. RSS often presents with symptoms and in an advanced stage. Surgical treatment is the most commonly used and chemotherapy for advanced or recurrent treatment.
Topics: Hematuria; Humans; Kidney Neoplasms; Neoplasm Recurrence, Local; Oncogene Proteins, Fusion; Pain; Sarcoma, Synovial; Survival Rate
PubMed: 33704587
DOI: 10.1007/s11934-021-01038-w -
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