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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 -
International Journal of Pediatric... Dec 2020Sarcomas are a rare, diverse tumor class of mesenchymal origin affecting all age groups. Survival after diagnosis is influenced by disease site. To date, there are no... (Review)
Review
BACKGROUND
Sarcomas are a rare, diverse tumor class of mesenchymal origin affecting all age groups. Survival after diagnosis is influenced by disease site. To date, there are no analyses evaluating treatment of pediatric sarcoma within the larynx specifically.
METHODS
A structured literature review following PRISMA guidelines was preformed to identify case reports of pediatric (age 17 and younger) laryngeal sarcoma.
RESULTS
Twenty-nine case reports documenting 37 pediatric patients diagnosed with sarcoma in the larynx were identified since 1980. The majority of patients were male (79.4%). The most common histological subtypes were rhabdomyosarcoma (69.4%) and synovial sarcoma (19.4%). The supraglottis was the most common site of disease (62.1%) among laryngeal subsites. Only two patients were known to have succumbed to their disease. Overall survival was not statistically impacted by primary site of tumor, treatment strategy, histology or gender.
CONCLUSIONS
Soft tissue sarcoma is rarely found in the pediatric larynx. Patient and tumor characteristics studied were not shown to affect outcomes. Increased documenting of high-quality case reports is needed to advance understanding of this disease.
Topics: Adolescent; Child; Female; Humans; Laryngeal Neoplasms; Larynx; Male; Sarcoma; Sarcoma, Synovial; Soft Tissue Neoplasms
PubMed: 33120103
DOI: 10.1016/j.ijporl.2020.110471 -
Sarcoma 2020Synovial sarcoma can present morphologically in multiple forms, including biphasic and monophasic subtypes. As a result, the histological diagnosis can sometimes be... (Review)
Review
BACKGROUND
Synovial sarcoma can present morphologically in multiple forms, including biphasic and monophasic subtypes. As a result, the histological diagnosis can sometimes be challenging. Transducin-Like Enhancer 1 (TLE1) is a transcriptional corepressor that normally is involved in embryogenesis and hematopoiesis but is also expressed in certain tumors. This systematic review examines the potential role of TLE1 as a diagnostic biomarker for the synovial sarcoma. . A literature review and meta-analysis were conducted using the electronic databases Pubmed, the Cochrane Library, and Google Scholar. Thirteen studies met our eligibility criteria and were selected for in-depth analysis.
RESULTS
The mean sensitivity and specificity of TLE1 in detecting synovial sarcoma were 94% (95% CI 91%-97%) and 81% (95% CI 72%-91%), respectively, when all studies were aggregated together. The mean positive predictive value (PPV) of TLE1 was 75% (95% CI 62%-87%), whereas the negative predictive value (NPV) was 96% (95% CI 93%-98%).
CONCLUSION
TLE1 is a sensitive and specific marker for synovial sarcoma that can aid in its diagnosis. Due to its involvement in several relevant signaling pathways, TLE1 might have direct relevance to the pathophysiology of the disease.
PubMed: 32322158
DOI: 10.1155/2020/7192347 -
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 -
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 -
Ear, Nose, & Throat Journal Feb 2021Primary laryngeal synovial sarcoma is an extremely rare tumor predominantly affecting young adults. There are currently no well-defined guidelines to direct...
Primary laryngeal synovial sarcoma is an extremely rare tumor predominantly affecting young adults. There are currently no well-defined guidelines to direct investigation and management, and treatment is largely based on what is known for synovial sarcoma of the upper and lower limbs. This PROSPERO-registered study aims to review the diagnostic methods, treatment regimens, and survival outcomes for patients with synovial sarcoma of the larynx. A systematic search of databases Medline, Embase, SCOPUS, and Web of Science was undertaken in December 2017. The literature search identified 1031 potentially relevant studies, and after the deletion of duplicates and excluded papers, 98 full-text articles were screened. A total of 39 cases were reviewed from 32 studies in the data extraction. The average age at the time of laryngeal synovial sarcoma diagnosis was 32 years (range, 11-79 years). In all cases (n = 39), patients underwent wide surgical excision, with 20 patients requiring a partial or total laryngectomy. A total of 18 patients received adjuvant and 3 received neoadjuvant radiotherapy. Chemotherapy was used in 10 cases, with ifosfamide the most frequently used agent. There was considerable variability in the order and combinations of the abovementioned treatments. No clinicopathologic factors or treatment regimens were associated with improved overall survival or lower rate of recurrence. There is a paucity of literature and heterogeneity in clinical approaches to this highly aggressive sarcoma. Reporting of cases must be standardized and formal guidelines must be established to guide clinical management.
Topics: Adolescent; Adult; Aged; Antineoplastic Agents; Child; Diagnosis, Differential; Female; Humans; Ifosfamide; Laryngeal Neoplasms; Laryngectomy; Larynx; Male; Middle Aged; Neoadjuvant Therapy; Neoplasm Recurrence, Local; Sarcoma, Synovial; Treatment Outcome; Young Adult
PubMed: 31309846
DOI: 10.1177/0145561319850697 -
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 -
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 -
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 -
World Journal of Clinical Oncology Feb 2020Despite the fact that about one third of patients with primary localized extremity soft tissue sarcoma (eSTS) will develop metastatic disease, abdominal metastases (AM)... (Review)
Review
BACKGROUND
Despite the fact that about one third of patients with primary localized extremity soft tissue sarcoma (eSTS) will develop metastatic disease, abdominal metastases (AM) and retroperitoneal metastases (RM) constitute rare events. There is no clear consensus on how to achieve follow-up on patients with primary localized eSTS following curative resection, especially regarding the surveillance of potential AM/RM.
AIM
To systematically analyse incidence, diagnosis, treatment and outcome of AM/RM in eSTS patients.
METHODS
In this systematic review, 899 studies available in PubMed and published between 2000 and 2018 were screened, identifying 17 original articles focused on AM or RM in eSTS. Article selection was based on the PRISMA guidelines, using the search terms (abdominal metastasis AND soft tissue sarcoma) and (soft tissue sarcoma metastasis abdomen). All studies published between January 1, 2000 and December 31, 2018 were screened. Further articles were identified by cross-searching article references, with the final search date being February 18, 2019. Due to limited data and the different reporting techniques used, the present review focused on descriptive analysis of the included studies.
RESULTS
Of the 17 studies included, six original articles reported on incidence ± diagnosis, therapy and outcome in AM and RM, whilst three original and eight case reports focused on diagnostic pathway, therapeutic procedures or outcomes without allowing conclusions regarding incidence of AM and RM. According to the former six studies, incidence of AM ranged from 0.9%-5.6% in patients with miscellaneous histological subtypes, and up to 12.1% in patients with myxoid liposarcoma. The most common histological subtypes that developed AM or RM were (myxoid) liposarcoma and leiomyosarcoma, but also rare subtypes such as epithelioid sarcoma, myxofibrosarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumour had been reported to develop AM/RM. Surgery for AM/RM was performed in five of eight case-reports (62.5%) and in 20.8%-100.0% of original articles. In particular, patients with hepatic metastases undergoing metastasectomy had a survival benefit compared to patients treated with chemotherapy or best supportive care (> 3 years < 6 mo).
CONCLUSION
Patients with eSTS should undergo surveillance with abdominal ultrasonography/computed tomography, or even whole-body-magnetic resonance imaging to detect AM/RM at an early stage.
PubMed: 32133276
DOI: 10.5306/wjco.v11.i2.74