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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 -
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 -
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 -
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 -
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 -
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 -
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 -
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