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Cite as: Archiv EuroMedica. 2025. 15; 2. DOI 10.35630/2025/15/2.211

Received 11 March 2025;
Accepted 9 April 2025;
Published 15 April 2025

SYNOVIAL SARCOMA AMONG ADULTS: FROM EPIDEMIOLOGY TO CLINICAL PRESENTATION, CURRENT DIAGNOSTIC STANDARDS, TREATMENT METHODS AND PROGNOSIS

Jakub Bulski1 email orcid id logo, Filip Maj1 orcid id logo,
Karol Sornat1 orcid id logo, Agata Estreicher2 orcid id logo,
Anna Klasa3 orcid id logo, Aleksandra Sobaś1 orcid id logo,
Kamil Biedka2 orcid id logo, Oliwia Ziobro2 orcid id logo,
Katarzyna Błaszczyk2 orcid id logo

1Collegium Medicum, Jan Kochanowski University, Kielce, Poland
2Wroclaw Medical University, Wroclaw, Poland
3The University Hospital in Krakow, Krakow, Poland

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  jakub.bulski@icloud.com

ABSTRACT

Aim: This article aims to comprehensively analyse the available literature on synovial sarcoma (SS), focusing on its pathogenesis, epidemiology, clinical presentation, diagnostic strategies, treatment approaches, and emerging therapeutic research.

Methods: A systematic literature search was conducted in PubMed, ClinicalKey and Google Scholar using the keywords synovial sarcoma and soft tissue sarcoma. A total of 104 peer-reviewed articles published within 1914-2025 were selected based on relevance, methodological quality and contribution to the field.

Results: SS is a rare soft tissue sarcoma (STS), accounting for 2–4.2% of all STS cases. It predominantly affects adults aged 20–44 years, with 10% of cases occurring among children. The tumor most commonly arises in the extremities, particularly the lower limbs, and typically presents as a painless mass. Diagnosis relies on magnetic resonance imaging (MRI) followed by biopsy, histopathological analysis, and molecular testing for SYT-SSX fusion genes. The mainstay of treatment is en bloc surgical resection, with radiotherapy and chemotherapy (e.g., doxorubicin + ifosfamide) serving as adjunct therapies in select cases.

Conclusions: SS presents a significant clinical challenge due to its rarity, nonspecific symptoms, and aggressive nature. Early detection of tumors, particularly those <5 cm, is crucial for improving outcomes. Multidisciplinary management in specialized sarcoma centers is essential for optimizing treatment strategies. Further research is needed to explore targeted therapies, immunotherapy, and molecular-driven treatment approaches to improve long-term prognosis.

Keywords: synovial sarcoma, soft tissue sarcoma, diagnosis, treatment, prognostic factors, targeted therapy

INTRODUCTION

Sarcomas are a rare and heterogeneous group of malignant neoplasms originating from mesenchymal tissue, accounting for only 1% of all malignancies in the human population [1]. They are traditionally classified into bone sarcomas and soft tissue sarcomas (STS) [2]. Among STS, which have an estimated incidence of 3.6 per 100,000 people, synovial sarcoma (SS) represents a distinct entity with unique molecular and clinical characteristics [3].

The World Health Organization (WHO) Classification of Tumours of Soft Tissue and Bone (4th edition) recognizes over 100 histological subtypes of STS, including SS, which is the focus of this article. [4]. The term synovial sarcoma was first introduced by Jones and Whitman in 1914 [5]. Initially, SS was believed to arise from the synovial membrane; however, subsequent research has demonstrated that its true origin lies in primitive mesenchymal tissue, with no direct relation to synovial structures [6].

A major breakthrough in understanding SS occurred in the 1990s, with the discovery of its hallmark chromosomal translocation t(X;18) [7]. This translocation leads to the fusion of the SYT gene with either SSX1 or SSX2, forming the SYT-SSX fusion protein, which plays a critical role in tumorigenesis [8,9]. While this fusion protein is known to regulate gene transcription, its exact oncogenic mechanism remains incompletely understood [10].

Histologically, SS is classified into three subtypes:

Considering SS’s complex biology, diagnostic challenges, and aggressive nature, a comprehensive review of its epidemiology, clinical presentation, and current treatment strategies is essential. This article aims to analyse key aspects of SS, with particular focus on diagnostic imaging techniques, histopathological evaluation, and therapeutic approaches. Additionally, prognostic factors influencing treatment outcomes will be discussed based on the latest research and guidelines.

MATERIALS AND METHODS

To conduct a thorough evaluation of the available data, we performed a literature search in the PubMed, Clinical Key, and Google Scholar databases using the keywords synovial sarcoma and soft tissue sarcoma. Based on the search results, we selected 104 peer-reviewed articles published within 1914-2025 that, in our opinion, most accurately describe the issue under investigation and provide valuable new insights into the topic.

RESULTS

EPIDEMIOLOGY AND LOCALIZATION

Among patients suffered from STS, depending on the sources, SS accounts only for 2% to 4.2% [3,12]. It is significant that the incidence of SS has been steadily increasing, from 0.906 per 1,000,000 people in the population in 1984 to 1.548 in 2012 [13]. Approximately 40% of SS cases involve patients aged 20 to 44, while another high-risk group consists of individuals aged 45 to 64. It is worth noting that among all patients diagnosed with SS, those under the age of 19 account for less than 10% [14]. Although SS is rare, it represents the most common non-rhabdomyosarcoma STS in the pediatric population [15].

In terms of localization, SS most frequently occurs in the limbs, with the primary tumor site commonly found in the distal regions of the lower extremities [16,17]. In rare cases, the primary lesion may be located within the joint cavity [18–20]. Less common than extremities locations include the trunk, head and neck, and chest [21,22]. However, it is important to note that SS can also occur in locations not typically recognized as primary sites for this tumor. Current literature includes approximately 70 documented cases of SS originating within the gastrointestinal tract. Among these, a study by Zhang et al. described a rare case of rectal SS presenting with rectal bleeding [23]. Another case report by Yalcin et al. described a 13-year-old boy with SS localized in the tonsil, further highlighting the diverse and atypical presentation sites of this malignancy [24].

CLINICAL PRESENTATION

The clinical presentation of SS largely depends on the tumor's location and stage of progression [25]. The most common clinical symptom observed in patients is the appearance of a painless mass at the site of the primary lesion [26]. It is important to consider that a significant proportion of patients do not present with this symptom. In a study of pediatric patients with SS Chotel et al. reported that 30.3% of participants did not exhibit any kind of visible mass. This study, however, was limited by a small sample size of 35 cases [27]. In a significant number of cases, the appearance of visible changes in the limbs is preceded by pain unrelated to injury at the site of the primary lesion, which should clearly prompt clinicians to consider a diagnosis of SS. De Silva et al. reported that in approximately 30% of SS patients, pain occurs before the appearance of tissue swelling or a palpable mass. In contrast, this symptom was present in only 3,6% of patients with other sarcomas (p < 0.001) [28]. In cases of periarticular localization, the tumor may cause a limitation in joint mobility [27]. As previously mentioned, atypical localization of SS can lead to unusual symptoms. Steinstraesser et al. reported the case of a 31-year-old man who presented to the hospital with classic symptoms of carpal tunnel syndrome lasting for three months. During surgery, a 2,5 cm mass was found within the carpal tunnel, which, after removal and pathological examination, was identified as SS [29]. The nonspecific symptoms and diagnostic challenges associated with SS are well illustrated by the case of a 39-year-old woman described by Hatano et al., who, despite clinical symptoms and multiple hospitalizations, remained without a correct diagnosis of SS for approximately 20 years [30].

More than one-third of patients with SS develop distant metastases [31]. The authors of the METASYN study conducted by the French Sarcoma Group demonstrated that among these individuals, the lungs are the most common metastatic site, accounting for 76,1% of cases, followed by lymph nodes (5.9%), pleura (5.1%), bones (4.3%), peritoneum (2.9%), and liver (1.6%) [32]. Krieg et al. report that metastases typically appear 5.7 years after diagnosis, but some cases occur more than 10 years later. The authors recommend that follow-up care for these patients be extended beyond 10 years [33].

DIAGNOSTTIC METHODS

The initial imaging diagnosis of SS, like other STS, should begin with a conventional X-ray and ultrasound examination of the suspicious area [34].

X-ray

The radiological features typically associated with SS include a soft tissue mass (67%), soft tissue calcifications (20%), and bony erosion (20%) [35].The main radiographic feature suggesting SS is the presence of a mineralized mass near, but not within, a joint, particularly in a young adult [36].

Ultrasound

Ultrasound is a valuable imaging modality in the initial diagnostic evaluation of soft tissue masses. It allows for the differentiation between fluid-filled lesions, for which further diagnostic work-up is typically not required, and solid masses. Additionally, ultrasound plays a role in guiding biopsy procedures, ensuring precise tissue sampling from the tumor [37,38].

Computed tomography (CT)

On CT, SS appears as a hypodense mass compared to adjacent muscle tissue [39,40]. Calcifications are present in approximately 30% of cases, most commonly at the tumor's periphery [36,40]. However, a study by Wilkerson et al. reported that calcifications were present in up to 62.5% of SS cases [41]. The authors themselves acknowledged a limitation of their study—namely, the small sample size (29 participants). In addition to hypodensity and calcifications, contrast enhancement is another critical imaging feature that may indicate the presence of SS. In a retrospective analysis of SS patient cases, Marzano et al. indicated that heterogeneous tumor tissue enhancement is present in up to 90% of cases [42]. Among the less characteristic features of SS, Wang et al. also mention the presence of hemorrhagic and cystic foci [40]. However, these features are not always present, and tumors smaller than 5 cm, with well-defined margins and a relatively slow growth rate, are often misinterpreted as benign lesions [36].

Magnetic resonance imaging (MRI)

MRI remains the preferred diagnostic method for evaluating soft tissue tumors, including SS. MRI enables high-resolution visualization of the tumor in relation to adjacent structures, allows for precise assessment of local tumor extent, and plays a crucial role in postoperative surveillance [43–45]. SS typically presents as a well-defined, oval or multilobulated mass on MRI. In T1-weighted (T1W) sequences, 50% of cases demonstrate a hypointense signal, while the remaining 50% exhibit an isointense signal relative to adjacent muscle tissue [46]. However, in certain cases, the presence of necrotic tissue or fluid-filled cysts within the tumor may result in hyperintensity in this sequence as well [44]. In relation to T2-weighted (T2W) sequences, Sedaghat et al. reported data indicating that SS consistently appears hyperintense compared to adjacent muscle tissue in all analyzed cases [47]. These findings align with the results of Ashikyan et al., who similarly observed hyperintensity of SS in T2W sequences across all studied cases. Furthermore, among the examined tumors, those containing septations or surrounded by a rim exhibited hypointensity relative to the predominant tumor mass in all cases. Notably, unequivocal results were not observed in T1W sequences. Rim characteristics were hypointense in 71% and hyperintense in 29% of cases, while septation characteristics were hypointense in 78% and hyperintense in 22% of cases [48]. The 'triple sign' is a characteristic MRI finding of SS, first described by Jones et al. in 1993 [49]. It refers to the simultaneous presence of:

This finding indicates significant heterogeneity within the tumor. However, it is not pathognomonic for SS [50]. The frequency of this sign in SS has been reported to range from 33% to 50% [39,48,51]. In T2W sequences, homogeneous tumors demonstrate contrast enhancement, with the exception of necrotic foci and fluid-filled spaces, if present, which show no increase in signal intensity following contrast administration [47]. Early arterial enhancement serves as a valuable diagnostic parameter in distinguishing benign lesions from sarcomas. This characteristic is observed in approximately 30% of benign lesions but is present in up to 70% of sarcomas, underscoring its potential utility in the differentiation process [52]. Furthermore, tumor enhancement within <7 seconds after arterial enhancement is a feature consistently observed in cases of SS [53]. This allows differentiating SS from other STS.

Biopsy

The 2021 ESMO-EURACAN-GENTURIS guidelines recommend multiple core needle biopsies (≥14-16 G needles) as the preferred method for diagnosing STS. Excisional biopsy is advised for superficial lesions located <3 cm from the skin [54]. Accurate histopathological classification relies on immunohistochemistry and molecular testing, particularly the detection of TLE1 and SS18-SSX fusion genes, which are highly specific for SS [55,56]. Fluorescence in situ hybridization (FISH) and reverse transcriptase–polymerase chain reaction (RT-PCR) remain the methods of choice for detecting the SS18-SSX mutation in the collected samples [57]. In cases where technical challenges hinder the collection of biopsy samples or where the tumor is located in atypical anatomical regions, ultrasound or CT-guided biopsy proves to be an effective approach. The utility of these techniques largely depends on the operator’s expertise, and they offer improved accuracy in tumor sampling [38,58].

Table 1. Diagnostic methods and their application in SS diagnosis.

Modality Findings in SS Key Diagnostic Role
X-ray Soft tissue mass (67%), calcifications (20%), bony erosion (20%) Initial screening
Ultrasound id vs. fluid-filled lesion; biopsy guidance Differentiates cystic from solid masses
CT Hypodense mass, peripheral calcifications (30-62.5%), contrast enhancement Helps assess extent and calcifications
MRI (T1W, T2W) T1W: Iso-/hypointense; T2W: Hyperintense with "triple sign" (33-50%) Gold standard for local assessment
Biopsy Core needle (≥14-16 G) preferred; SS18-SSX fusion gene Confirms diagnosis

TREATMENT OPTIONS

The therapeutic approach to SS encompasses surgical resection, chemotherapy, and radiotherapy as the primary modalities. In specific clinical scenarios, these methods are integrated into multimodal treatment protocols to enhance patient outcomes [59]. Selecting an appropriate center for the treatment of SS is paramount, and experts emphasize the importance of specialized institutions. These centers should be equipped with a multidisciplinary team, including pediatric/orthopedic surgeons, pathologists, radiologists, and clinical oncologists, all with extensive experience in diagnosing and managing STS. Furthermore, early referral of patients to such specialized centers is critical to optimize outcomes [60,61]. Specifically, any patient with a soft tissue lesion, whether superficial or deep, exceeding 5 cm in diameter, should be promptly referred to a reference center for STS treatment [54].

Surgery

International guidelines and scientific consensus recommend surgical excision as the treatment of choice for SS. This approach is particularly considered for patients with localized tumors without the presence of distant metastases [62,63]. According to the 2023 Consensus on surgical margin definition harmonization from the International Soft Tissue Sarcoma Consortium (INSTRuCT), the goal of surgery is radical excision with the achievement of microscopically tumor-free margins (R0 resection), while preserving the function and shape of the operated area. The entire excised tumor tissue, including the margin of healthy tissue and the biopsy needle tract, should be removed as one tissue block (en bloc resection) and sent for histopathological examination. In the case of SS adjacent to bone, the surrounding periosteum should be excised. A similar approach applies to tumors near the fascia, where the fascia should also be removed. If the resection is not radical (R1 or R2 margin), reoperation should be performed to excise the scar and the superficial and deep tissues left behind during the initial surgery [64]. In certain anatomical locations, achieving en bloc resection with an R0 margin may be extremely difficult. In these cases preoperative radiotherapy or chemotherapy may be justified to reduce tumor volume and make the surgery more feasible [65,66].

In selected cases, due to the location or advanced stage of the disease, amputation may be the best therapeutic option, allowing for local control and offering the greatest chance of cure. However, this method is generally used as a last resort, for example, in the case of some patients with relapsed SS [62,67]. Metastasis is more frequent in patients undergoing amputation, mainly due to factors like large tumor size and highly malignant histology. However, amputation itself is not an independent risk factor for distant metastasis [68].

In some cases of patients with distant metastases in the lungs, metastasectomy may be considered as a surgical treatment option. However, there is a lack of strong evidence clearly demonstrating a positive impact of this procedure on outcomes [69–71]. This underscores the need for further studies on the impact of metastasectomy on prognosis.

Radiotherapy (RT)

RT, in combination with surgical resection, represents a cornerstone in the treatment of SS for patients who meet specific eligibility criteria [22]. The NCCN Clinical Practice Guidelines in Oncology for Soft Tissue Sarcoma (Version 2.2018) recommend considering RT for patients with:

Preoperative RT remains essential for tumors larger than 5 cm, recurrent tumors, and those located near critical structures [54,62]. The standard dose for neoadjuvant external beam RT is 50 Gy (1.8-2.0 Gy per fraction), with additional postoperative doses for patients with R1 margins (16-18 Gy) and R2 margins (20-26 Gy). Adjuvant RT uses similar dosing, with patients having R0 margins receiving an additional 10-16 Gy on top of the initial 50 Gy [72]. For patients with regionally advanced disease, RT may be omitted if the postoperative histopathological examination confirms an R0 resection margin, particularly for tumors smaller than 5 cm [15,73]. For patients with distant metastases, the treatment strategy depends on the extent of metastatic disease. Solitary metastases may be managed with a combination of RT, chemotherapy, and metastasectomy. In cases of disseminated metastases, palliative RT should be considered [72]. Previous studies confirm the positive impact of RT on overall survival (OS) among patients with SS [74–76]. Additionally, Song et al. demonstrated a statistically significant improvement in progression-free survival (p=0.006) and 5-year local-recurrence-free survival (p=0.028) in patients who underwent adjuvant RT after surgery compared to those treated with surgery alone (77). These data highlight RT's significant impact on SS treatment.

Chemotherapy

Due to the relatively high sensitivity of SS to chemotherapy compared to other STS, it is often included in treatment strategies, both in the neoadjuvant and adjuvant settings [78]. This results from the high grade of SS cells and its faster growth compared to less chemosensitive, intermediate grade malignant STS, such as schwannomas or leiomyosarcomas[79]. The standard first-line drug is doxorubicin, often combined with ifosfamide [62]. Spurrell et al. showed that this combination treatment is associated with a better response rate (58,6%) compared to doxorubicin alone (25%) or ifosfamide alone (25%) [70]. High-risk patients should be considered for chemotherapy, particularly those with:

Scientific evidence on the impact of chemotherapy on the prognosis of adult patients with SS remains conflicting, indicating that it should be used only in specific cases [22,80–83]. Currently, pazopanib, a tyrosine kinase inhibitor, remains the only drug beyond classical chemotherapeutics used in patients with SS (84). Ongoing research focuses on developing new drugs, particularly immunotherapy, to improve prognosis and reduce side effects of conventional therapies in SS patients [85–88].

PROGNOSIS

Patient-specific factors

The prognosis among patients with SS is variable and depends on a combination of patient-specific factors and tumor-related characteristics. An analysis of the SEER database conducted by Aytekin et al. revealed that among patients with SS, the one-, five-, ten-, and twenty-year survival rates were 87.3%, 59.4%, 50.8%, and 42.8%, respectively, with a median OS of 138 months. However, age-based subgroup analysis showed a significant difference in survival. Among patients aged ≥35 years, median OS was only 60 months, which was statistically significantly lower than the 200 months observed in patients under 35 years of age (p<0.001) [89]. These data confirm that age ≥35 years of age is an independent prognostic factor for unfavorable outcome in patients with SS. The better prognosis in younger patients is confirmed by Vlenterie et al., who found the highest OS rate in those under 18 years of age [90]. Male gender and Black race are additional factors associated with a worse prognosis [75]. However, the authors do not explain the underlying mechanisms of this poorer outcome in these patient groups. Interestingly, Sultan et al. observed that these factors did not significantly affect prognosis in the pediatric population. However, the authors themselves acknowledge that these findings may be influenced by the relatively small number of children included in the study [91]. A particularly unfavorable prognostic factor is the presence of distant metastases at the time of diagnosis [33]. Smolle et al. demonstrated that in this patient group, the 5-year cancer-specific survival is 22.6% [92]. This represents a nearly threefold worse outcome compared to patients without distant metastases.

Tumor characteristics

Tumor-related prognostic features include the size of the primary lesion, histological subtype, tumor grade and negative surgical margins. For STS, a tumor size of 5 cm serves as the threshold for classifying a lesion as T1 according to the TNM classification. Tumors larger than this diameter are categorized as T2 or higher [93]. Kang et al. demonstrated that in cases of STS, a tumor size exceeding 5 cm is associated with a worse prognosis compared to smaller lesions. The disease-specific survival at 5 years was 87.4% in the T1 group versus 74.9% in the T2 group (p=0.001) (94). Research on SS indicates that, like other STS, larger tumor size at diagnosis is associated with a worse prognosis [92,95–97]. The histological subtype of the tumor is a significant factor in assessing prognosis. Studies indicate that the biphasic subtype has the most favorable outcomes among all histological variants of SS. Conversely, the epithelioid subtype is associated with the poorest OS [13,89]. These findings are corroborated by a study by Xiong et al., which analyzed 1692 patients and demonstrated that the five- and ten-year survival rates varied by histological subtype: biphasic subtype (69%, 60%), monophasic subtype (59%, 49%), and epithelioid subtype (32%, 26%) [11]. These data confirm the superior prognosis for the biphasic SS subtype compared to other variants. Fice et al. also highlighted the significant impact of histological grade on prognosis, reporting an metastasis-free survival (MFS) rate of 86,5% for G2 tumors, while G3 tumors had a markedly lower MFS rate of just 50% (p=0,026) [98]. The negative impact of higher histological grades on MFS has also been confirmed by Trassard et al. [99].

Radicality of surgery

The radicality of surgical resection is another significant factor influencing prognosis. Numerous studies have shown that incomplete resection (R1/R2) significantly worsens overall survival [22,32,100]. The impact of this factor on the occurrence of distant metastases in the future varies depending on the source, with Sacchetti et al. demonstrating, after performing a multivariate analysis, that its effect is just above statistical significance, despite showing a significant influence on recurrence-free survival in univariate analyses [77,100,101]. A multicenter study by Trovik et al. involving 559 patients and a systematic review by Fanfan et al. including 123 patients found no significant impact of resection radicality on the development of distant metastases [102,103]. These studies did not differentiate between STS types, highlighting the need for a meta-analysis focused on the impact of resection on distant metastasis development in SS patients. Incomplete resection also negatively affects local recurrence outcomes [104]. These findings emphasize the critical role of achieving an R0 margin in resection for prognosis.

CONCLUSION

  1. Synovial sarcoma (SS) presents a significant clinical challenge due to its high aggressiveness, complex diagnosis, and limited therapeutic options. Early diagnosis and referral of patients to specialized centers with multidisciplinary teams are key factors for successful treatment.
  2. Surgical treatment remains the primary therapeutic approach for SS. Achieving an R0 resection (microscopically tumor-free margins) is critical for improving overall survival and reducing the risk of local recurrence. In cases where radical surgery is not feasible, neoadjuvant chemotherapy and/or radiotherapy may be justified.
  3. Radiotherapy is an essential component of combined treatment, particularly for patients with high-grade tumors, positive surgical margins (R1/R2), and large tumors (>5 cm). It contributes to reducing the risk of local recurrence and improving overall survival.
  4. Chemotherapy is used in neoadjuvant or adjuvant settings, especially for high-risk patients (G2-G3 tumors, >5 cm, R1/R2 resection, metastases). However, its impact on long-term survival remains controversial, and treatment decisions should be individualized.
  5. The prognosis of SS depends on multiple factors, including patient age, tumor size, histological subtype, tumor grade, and the radicality of surgical intervention. Younger patients (<35 years) have significantly better overall survival rates, whereas the presence of distant metastases at diagnosis drastically worsens the prognosis.
  6. Targeted therapy and immunotherapy represent promising directions in SS treatment, with the potential to revolutionize management, particularly for patients with advanced and treatment-resistant forms of the disease. Further research is necessary to develop more effective treatment strategies.
  7. The key to successful SS treatment is early diagnosis and a comprehensive therapeutic approach. Patients should be treated in specialized centers with the necessary expertise and resources. The development of personalized treatment strategies based on molecular tumor profiling remains a crucial goal in modern oncology.

DISCLOSURES

Funding Statement. This Research received no external funding.

Conflicts of Interests: The authors declare no conflict of interest.

REFERENCES

  1. Grünewald TG, Alonso M, Avnet S, Banito A, Burdach S, Cidre?Aranaz F, et al. Sarcoma treatment in the era of molecular medicine. EMBO Mol Med. 2020 Nov 6;12(11):e11131. DOI: 10.15252/emmm.201911131
  2. Clemente O, Ottaiano A, Di Lorenzo G, Bracigliano A, Lamia S, Cannella L, et al. Is immunotherapy in the future of therapeutic management of sarcomas? J Transl Med. 2021 Dec;19(1):173. DOI: 10.1186/s12967-021-02829-y
  3. Ducimetière F, Lurkin A, Ranchère-Vince D, Decouvelaere AV, Péoc’h M, Istier L, et al. Incidence of sarcoma histotypes and molecular subtypes in a prospective epidemiological study with central pathology review and molecular testing. PloS One. 2011;6(8):e20294. DOI: 10.1371/journal.pone.0020294
  4. Fletcher CDM, International Agency for Research on Cancer, editors. WHO classification of tumours of soft tissue and bone: reflects the views of a working group that convened for a consensus and editorial meeting at the University of Zurich, Switzerland, 18-20 april 2012. 4. ed. Lyon: Internat. Agency for Research on Cancer; 2013. 468 p. (World Health Organization classification of tumours).
  5. Jones SF, Whitman RC. PRIMARY SARCOMA OF THE LOWER END OF THE FEMUR INVOLVING THE SYNOVIAL MEMBRANE WITH A CRITICAL REVIEW OF THE LITERATURE OF SYNOVIAL SARCOMA REPORT OF ONE CASE WITH COMPLETE PATHOLOGICAL AND RADIOGRAPHIC EXAMINATION: Ann Surg. 1914 Oct;60(4):440–50. DOI: 10.1097/00000658-191410000-00002
  6. Robbins PF, Morgan RA, Feldman SA, Yang JC, Sherry RM, Dudley ME, et al. Tumor Regression in Patients With Metastatic Synovial Cell Sarcoma and Melanoma Using Genetically Engineered Lymphocytes Reactive With NY-ESO-1. J Clin Oncol. 2011 Mar 1;29(7):917–24. DOI: 10.1200/JCO.2010.32.2537
  7. Brand RA. 50 Years Ago in CORR®: Synovial Sarcoma Kirk J. Anderson, MD and Orliss Wildermuth, MD CORR 1961;29:55-70. Clin Orthop. 2013 Mar;471(3):1064–6.
  8. Clark J, Rocques PJ, Crew AJ, Gill S, Shipley J, Chan AML, et al. Identification of novel genes, SYT and SSX, involved in the t(X;18)(p11.2;q11.2) translocation found in human synovial sarcoma. Nat Genet. 1994 Aug;7(4):502–8.
  9. Ladanyi M. Fusions of the SYT and SSX genes in synovial sarcoma. Oncogene. 2001 Sep 10;20(40):5755–62.
  10. Feng X, Huang YL, Zhang Z, Wang N, Yao Q, Pang LJ, et al. The role of SYT-SSX fusion gene in tumorigenesis of synovial sarcoma. Pathol - Res Pract. 2021 Jun;222:153416. DOI: 10.1016/j.prp.2021.153416
  11. Xiong L, Chen Z, Zhou Y, Li H, Xiao T. The survival and prognosis analysis of synovial sarcoma subtypes: a Surveillance, Epidemiology, and End Results population-based analysis. Int Orthop. 2020 Dec;44(12):2779–86. DOI: 10.1007/s00264-020-04708-5
  12. Honoré C, Méeus P, Stoeckle E, Bonvalot S. Soft tissue sarcoma in France in 2015: Epidemiology, classification and organization of clinical care. J Visc Surg. 2015 Sep;152(4):223–30. DOI: 10.1016/j.jviscsurg.2015.05.001
  13. Wang S, Song R, Sun T, Hou B, Hong G, Mallampati S, et al. Survival changes in Patients with Synovial Sarcoma, 1983-2012. J Cancer. 2017;8(10):1759–68. doi: 10.7150/jca.17349
  14. Joseph N, St. Laurent S, Zheng S, Stirnadel-Farrant H, Dharmani C. Epidemiology of synovial sarcoma in EU28 countries. Ann Oncol. 2019 Oct;30:v706–7.
  15. Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, et al. A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children’s Oncology Group prospective study. Lancet Oncol. 2020 Jan;21(1):145–61. DOI: 10.1016/S1470-2045(19)30672-2
  16. Ferrari A, Gronchi A, Casanova M, Meazza C, Gandola L, Collini P, et al. Synovial sarcoma: A retrospective analysis of 271 patients of all ages treated at a single institution. Cancer. 2004 Aug;101(3):627–34. DOI: 10.1002/cncr.20386
  17. Cebada Chaparro E. Synovial Sarcoma: Imaging findings and prognostic features. 2018;1856 words.
  18. Ishida T, Iijima T, Moriyama S, Kitagawa T, Machinami R, Nakamura C. Intra-articular calcifying synovial sarcoma mimicking synovial chondromatosis. Skeletal Radiol. 1996 Nov 18;25(8):766–9. DOI: 10.1007/s002560050176
  19. Asiri Y, Almushayqih M, Alshamlan N, Alshaalan M. Case report of intra-articular synovial sarcoma in the hip joint. Radiol Case Rep. 2020 Aug;15(8):1256–60. DOI: 10.1016/j.radcr.2020.05.026
  20. Saito K, Kawabata Y, Kobayashi N, Iwashita H, Kato I, Otani M, et al. A rare case of intra-articular synovial sarcoma of the hip joint: a case report with intra-articular findings via hip arthroscopy. J Surg Case Rep. 2023 Feb 1;2023(2):rjad066. DOI: 10.1093/jscr/rjad066
  21. Canter RJ, Qin LX, Maki RG, Brennan MF, Ladanyi M, Singer S. A Synovial Sarcoma-Specific Preoperative Nomogram Supports a Survival Benefit to Ifosfamide-Based Chemotherapy and Improves Risk Stratification for Patients. Clin Cancer Res. 2008 Dec 15;14(24):8191–7. DOI: 10.1158/1078-0432.CCR-08-0843
  22. Italiano A, Penel N, Robin YM, Bui B, Le Cesne A, Piperno-Neumann S, et al. Neo/adjuvant chemotherapy does not improve outcome in resected primary synovial sarcoma: a study of the French Sarcoma Group. Ann Oncol. 2009 Mar;20(3):425–30. DOI: 10.1093/annonc/mdn678
  23. Zhang J, Findeis SK, Lang BJ, Ogola GO, Agarwal A. Primary rectal monophasic synovial sarcoma. Bayl Univ Med Cent Proc. 2021 Jul 4;34(4):512–6. DOI: 10.1080/08998280.2021.1902191
  24. Yalcin K, Tuysuz G, Genc M, Ozbudak IH, Derin AT, Karaali K, et al. Pediatric tonsillar synovial sarcoma- very rare localization: a case report and review of the literature. Turk J Pathol [Internet]. 2019 [cited 2024 Oct 28]; Available from: http://www.turkjpath.org/doi.php?doi=10.5146/tjpath.2018.01449
  25. Li C, Krasniqi F, Donners R, Kettelhack C, Krieg AH. Synovial sarcoma: the misdiagnosed sarcoma. EFORT Open Rev. 2024 Mar 1;9(3):190–201. DOI: 10.1530/EOR-23-0193
  26. Ichinose H, Wickstrom JK, Hoerner HE, Derbes VL. The early clinical presentation of synovial sarcoma. Clin Orthop. 1979;(142):185–9.
  27. Chotel F, Unnithan A, Chandrasekar CR, Parot R, Jeys L, Grimer RJ. Variability in the presentation of synovial sarcoma in children: A PLEA FOR GREATER AWARENESS. J Bone Joint Surg Br. 2008 Aug;90-B(8):1090–6. DOI: 10.1302/0301-620X.90B8.19815
  28. De Silva MVC, Barrett A, Reid R. Premonitory Pain Preceding Swelling: A Distinctive ClinicalPresentation of Synovial Sarcoma which may Prompt Early Detection. Sarcoma. 2003 Jan;7(3–4):131–5. DOI: 10.1080/13577140310001644788
  29. Steinstraesser L, Agarwal R, Stricker I, Steinau HU, Al-Benna S. Biphasic Synovial Sarcoma of the Extremity: Quadruple Approach of Isolated Limb Perfusion, Surgical Ablation, Adipofascial Perforator Flap and Radiation to Avoid Amputation. Case Rep Oncol. 2011 Apr 6;4(1):222–8. DOI: 10.1159/000327845
  30. Hatano H, Yamagishi T, Yanabashi K, Ogose A. Underlying synovial sarcoma undiagnosed for more than 20 years in a patient with regional pain: a case report. Skeletal Radiol [Internet]. 2024 May 10 [cited 2024 Nov 6]; Available from: https://link.springer.com/10.1007/s00256-024-04701-8
  31. Pokras S, Tseng WY, Espirito JL, Beeks A, Culver K, Nadler E. Treatment patterns and outcomes in metastatic synovial sarcoma: a real-world study in the US oncology network. Future Oncol. 2022 Oct;18(32):3637–50. DOI: 10.2217/fon-2022-0477
  32. Moreau-Bachelard C, Campion L, Toulmonde M, Le Cesne A, Brahmi M, Italiano A, et al. Patterns of care and outcomes of 417 patients with METAstatic SYNovial sarcoma (METASYN): real-life data from the French Sarcoma Group (FSG). ESMO Open. 2022 Apr;7(2):100402. DOI: 10.1016/j.esmoop.2022.100402
  33. Krieg AH, Hefti F, Speth BM, Jundt G, Guillou L, Exner UG, et al. Synovial sarcomas usually metastasize after >5 years: a multicenter retrospective analysis with minimum follow-up of 10 years for survivors. Ann Oncol. 2011 Feb;22(2):458–67. DOI: 10.1093/annonc/mdq394
  34. Crombé A, Kind M, Fadli D, Miceli M, Linck PA, Bianchi G, et al. Soft-tissue sarcoma in adults: Imaging appearances, pitfalls and diagnostic algorithms. Diagn Interv Imaging. 2023 May;104(5):207–20. DOI: 10.1016/j.diii.2022.12.001
  35. Horowitz AL, Resnick D, Caird Watson R. The roentgen features of synovial sarcomas. Clin Radiol. 1973 Jan;24(4):481–4. DOI: 10.1016/s0009-9260(73)80158-8
  36. Murphey MD, Gibson MS, Jennings BT, Crespo-Rodríguez AM, Fanburg-Smith J, Gajewski DA. Imaging of Synovial Sarcoma with Radiologic-Pathologic Correlation. RadioGraphics. 2006 Sep;26(5):1543–65. DOI: 10.1148/rg.265065084
  37. Bixby SD, Hettmer S, Taylor GA, Voss SD. Synovial Sarcoma in Children: Imaging Features and Common Benign Mimics. Am J Roentgenol. 2010 Oct;195(4):1026–32. DOI: 10.2214/AJR.10.4348
  38. Cernakova M, Hobusch GM, Amann G, Funovics PT, Windhager R, Panotopoulos J. Diagnostic accuracy of ultrasound-guided core needle biopsy versus incisional biopsy in soft tissue sarcoma: an institutional experience. Sci Rep. 2021 Sep 8;11(1):17832. DOI: 10.1038/s41598-021-96953-w
  39. Baheti AD, Tirumani SH, Sewatkar R, Shinagare AB, Hornick JL, Ramaiya NH, et al. Imaging features of primary and metastatic extremity synovial sarcoma: a single institute experience of 78 patients. Br J Radiol. 2015 Feb;88(1046):20140608. DOI: 10.1259/bjr.20140608
  40. Wang DJ, Alwafi L, Pritchett SL, Wehrli BM, Spouge ARI. The Imaging Spectrum of Synovial Sarcomas: A Pictorial Review From a Single-Centre Tertiary Referral Institution. Can Assoc Radiol J. 2021 Aug;72(3):470–82. DOI: 10.1177/0846537119899284
  41. Wilkerson BW, Crim JR, Hung M, Layfield LJ. Characterization of Synovial Sarcoma Calcification. Am J Roentgenol. 2012 Dec;199(6):W730–4. DOI: 10.2214/AJR.11.7342
  42. Marzano L, Failoni S, Gallazzi M, Garbagna P. The role of diagnostic imaging in synovial sarcoma. Our experience. Radiol Med (Torino). 2004;107(5–6):533–40.
  43. Vibhakar AM, Cassels JA, Botchu R, Rennie WJ, Shah A. Imaging update on soft tissue sarcoma. J Clin Orthop Trauma. 2021 Nov;22:101568. DOI: 10.1016/j.jcot.2021.101568
  44. Liang C, Mao H, Tan J, Ji Y, Sun F, Dou W, et al. Synovial sarcoma: Magnetic resonance and computed tomography imaging features and differential diagnostic considerations. Oncol Lett. 2015 Feb;9(2):661–6. DOI: 10.3892/ol.2014.2774
  45. Angelini A, Biz C, Cerchiaro M, Longhi V, Ruggieri P. Malignant Bone and Soft Tissue Lesions of the Foot. J Clin Med. 2023 Apr 21;12(8):3038. DOI: 10.3390/jcm12083038
  46. Tordjman M, Honoré C, Crombé A, Bouhamama A, Feydy A, Dercle L, et al. Prognostic factors of the synovial sarcoma of the extremities: imaging does matter. Eur Radiol. 2022 Aug 18;33(2):1162–73. DOI: 10.1007/s00330-022-09049-y
  47. Sedaghat M, Sedaghat S. Primary synovial sarcoma on MRI – a case series and review of the literature. Pol J Radiol. 2023 Jul 10;88:325–30. DOI: 10.5114/pjr.2023.130048
  48. Ashikyan O, Bradshaw SB, Dettori NJ, Hwang H, Chhabra A. Conventional and advanced MR imaging insights of synovial sarcoma. Clin Imaging. 2021 Aug;76:149–55. DOI: 10.1016/j.clinimag.2021.02.010
  49. Jones BC, Sundaram M, Kransdorf MJ. Synovial sarcoma: MR imaging findings in 34 patients. Am J Roentgenol. 1993 Oct;161(4):827–30. DOI: 10.2214/ajr.161.4.8396848
  50. Sqalli Houssaini A, Saouab R, Essetti S, En-nouali H, El Fenni J, Boui M. CT and MR imaging of a huge retroperitoneal synovial sarcoma: A case report. Radiol Case Rep. 2025 Jan;20(1):205–9. DOI: 10.1016/j.radcr.2024.09.151
  51. Chen JY, Liu QY, Ye RX, Zhong JL, Liang BL. [Correlation of MR imaging features and histopathology of synovial sarcoma]. Ai Zheng Aizheng Chin J Cancer. 2005 Jan;24(1):87–90.
  52. Zadeh FS, Pooyan A, Alipour E, Hosseini N, Thurlow PC, Del Grande F, et al. Dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in differentiation of soft tissue sarcoma from benign lesions: a systematic review of literature. Skeletal Radiol. 2024 Jul;53(7):1343–57. DOI: 10.1007/s00256-024-04598-3
  53. Van Rijswijk CSP, Hogendoorn PCW, Taminiau AHM, Bloem JL. Synovial sarcoma: dynamic contrast-enhanced MR imaging features. Skeletal Radiol. 2001 Feb 12;30(1):25–30. DOI: 10.1007/s002560000295
  54. Gronchi A, Miah AB, Dei Tos AP, Abecassis N, Bajpai J, Bauer S, et al. Soft tissue and visceral sarcomas: ESMO–EURACAN–GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up?. Ann Oncol. 2021 Nov;32(11):1348–65. DOI: 10.1016/j.annonc.2021.07.006
  55. Errani C, Traina F, Perna F, Calamelli C, Faldini C. Current Concepts in the Biopsy of Musculoskeletal Tumors. Damron TA, Pflugmacher R, Wahlstrom O, editors. Sci World J. 2013 Jan;2013(1):538152. doi: 10.1155/2013/538152
  56. Georgescu AC, Georgescu TA, Duca-Barbu SA, Pop LG, Toader DO, Suciu N, et al. A Comprehensive Review of TRPS1 as a Diagnostic Immunohistochemical Marker for Primary Breast Carcinoma: Latest Insights and Diagnostic Pitfalls. Cancers. 2024 Oct 23;16(21):3568. DOI: 10.3390/cancers16213568
  57. Amary MFC, Berisha F, Bernardi FDC, Herbert A, James M, Reis-Filho JS, et al. Detection of SS18-SSX fusion transcripts in formalin-fixed paraffin-embedded neoplasms: analysis of conventional RT-PCR, qRT-PCR and dual color FISH as diagnostic tools for synovial sarcoma. Mod Pathol. 2007 Apr;20(4):482–96. DOI: 10.1038/modpathol.3800761
  58. Rimondi E, Rossi G, Bartalena T, Ciminari R, Alberghini M, Ruggieri P, et al. Percutaneous CT-guided biopsy of the musculoskeletal system: Results of 2027 cases. Eur J Radiol. 2011 Jan;77(1):34–42. DOI: 10.1016/j.ejrad.2010.06.055
  59. Zeng Z, Yao H, Lv D, Jin Q, Bian Y, Zou Y, et al. Multimodal Risk-Adapted Treatment in Surgical Patients With Synovial Sarcoma: A Preoperative Nomogram-Guided Adjuvant Treatment Strategy. Front Surg. 2020 Dec 21;7:579726. DOI: 10.3389/fsurg.2020.579726
  60. Casali PG, Abecassis N, Bauer S, Biagini R, Bielack S, Bonvalot S, et al. Soft tissue and visceral sarcomas: ESMO–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct;29:iv51–67. DOI: 10.1093/annonc/mdy096
  61. Gouin F, Stoeckle E, Honoré C, Ropars M, Jafari M, Mattei JC, et al. Overall survival in patients with re-excision of positive microscopic margins of limb and trunk wall soft tissue sarcoma operated outside of a reference center: a nationwide cohort analysis. BMC Cancer. 2022 Oct 3;22(1):1034. DOI: 10.1186/s12885-022-10121-5
  62. Hayes AJ, Nixon IF, Strauss DC, Seddon BM, Desai A, Benson C, et al. UK guidelines for the management of soft tissue sarcomas. Br J Cancer [Internet]. 2024 May 11 [cited 2024 Nov 18]; Available from: https://www.nature.com/articles/s41416-024-02674-y
  63. Blay JY, Soibinet P, Penel N, Bompas E, Duffaud F, Stoeckle E, et al. Improved survival using specialized multidisciplinary board in sarcoma patients. Ann Oncol. 2017 Nov;28(11):2852–9. DOI: 10.1093/annonc/mdx484
  64. Sparber-Sauer M, Ferrari A, Spunt SL, Vokuhl C, Casey D, Lautz TB, et al. The significance of margins in pediatric Non-Rhabdomyosarcoma soft tissue sarcomas: Consensus on surgical margin definition harmonization from the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT). Cancer Med. 2023 May;12(10):11719–30. doi: 10.1002/cam4.5671
  65. Dangoor A, Seddon B, Gerrand C, Grimer R, Whelan J, Judson I. UK guidelines for the management of soft tissue sarcomas. Clin Sarcoma Res. 2016 Dec;6(1):20. DOI: 10.1186/s13569-016-0060-4
  66. Le Grange F, Cassoni AM, Seddon BM. Tumour volume changes following pre-operative radiotherapy in borderline resectable limb and trunk soft tissue sarcoma. Eur J Surg Oncol EJSO. 2014 Apr;40(4):394–401. DOI: 10.1016/j.ejso.2014.01.011
  67. Ferrari A, Berlanga P, Gatz SA, Schoot RA, Van Noesel MM, Hovsepyan S, et al. Treatment at Relapse for Synovial Sarcoma of Children, Adolescents and Young Adults: From the State of Art to Future Clinical Perspectives. Cancer Manag Res. 2023 Oct;Volume 15:1183–96. DOI: 10.2147/CMAR.S404371
  68. Ghert MA, Abudu A, Driver N, Davis AM, Griffin AM, Pearce D, et al. The Indications for and the Prognostic Significance of Amputation as the Primary Surgical Procedure for Localized Soft Tissue Sarcoma of the Extremity. Ann Surg Oncol. 2005 Jan;12(1):10–7. DOI: 10.1007/s10434-004-1171-3
  69. Wang Y, Delisle M, Smith D, Alshamsan B, Srikanthan A. Metastasectomy in synovial sarcoma: A systematic review and meta-analysis. Eur J Surg Oncol. 2022 Sep;48(9):1901–10. DOI: 10.1016/j.ejso.2022.05.022
  70. Spurrell EL, Fisher C, Thomas JM, Judson IR. Prognostic factors in advanced synovial sarcoma: an analysis of 104 patients treated at the Royal Marsden Hospital. Ann Oncol. 2005 Mar;16(3):437–44. DOI: 10.1093/annonc/mdi082
  71. Tetta C, Montrone G, Longhi A, Rocca M, Londero F, Parise G, et al. Chemosensitivity of Lung Metastatic High-Grade Synovial Sarcoma. J Clin Med. 2021 Dec 18;10(24):5956. DOI: 10.3390/jcm10245956
  72. Von Mehren M, Randall RL, Benjamin RS, Boles S, Bui MM, Ganjoo KN, et al. Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2018 May;16(5):536–63. DOI: 10.6004/jnccn.2018.0025
  73. Hagiwara Y, Iwata S, Ogura K, Kawai A, Susa M, Morioka H, et al. Clinical analysis of multimodal treatment for localized synovial sarcoma: A multicenter retrospective study. J Orthop Sci. 2023 Jan;28(1):261–6. DOI: 10.1016/j.jos.2021.09.012
  74. Gingrich AA, Marrufo AS, Liu Y, Li CS, Darrow MA, Monjazeb AM, et al. Radiotherapy is Associated With Improved Survival in Patients With Synovial Sarcoma Undergoing Surgery: A National Cancer Database Analysis. J Surg Res. 2020 Nov;255:378–87. DOI: 10.1016/j.jss.2020.05.075
  75. Naing KW, Monjazeb AM, Li CS, Lee LY, Yang A, Borys D, et al. Perioperative radiotherapy is associated with improved survival among patients with synovial sarcoma: A SEER analysis: Radiotherapy and Synovial Sarcoma. J Surg Oncol. 2015 Feb;111(2):158–64. DOI: 10.1002/jso.23780
  76. Jami SA, Mobarak SA, Jiandang S, Xi Z, Tanvir MMS, Monilal SS. Clinical and strategic outcomes of metastatic synovial sarcoma on limb. Int J Health Sci. 2020;14(6):38–43.
  77. Song S, Park J, Kim HJ, Kim IH, Han I, Kim HS, et al. Effects of Adjuvant Radiotherapy in Patients With Synovial Sarcoma. Am J Clin Oncol. 2017 Jun;40(3):306–11. DOI: 10.1097/COC.0000000000000148
  78. Vlenterie M, Litière S, Rizzo E, Marréaud S, Judson I, Gelderblom H, et al. Outcome of chemotherapy in advanced synovial sarcoma patients: Review of 15 clinical trials from the European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group; setting a new landmark for studies in this entity. Eur J Cancer. 2016 May;58:62–72. DOI: 10.1016/j.ejca.2016.02.002
  79. Rosen G, Forscher C, Lowenbraun S, Eilber F, Eckardt J, Holmes C, et al. Synovial sarcoma. Uniform response of metastases to high dose ifosfamide. Cancer. 1994 May 15;73(10):2506–11. DOI: 10.1002/1097-0142(19940515)73:10<2506::aid-cncr2820731009>3.0.co;2-s
  80. Le Cesne A, Ouali M, Leahy MG, Santoro A, Hoekstra HJ, Hohenberger P, et al. Doxorubicin-based adjuvant chemotherapy in soft tissue sarcoma: pooled analysis of two STBSG-EORTC phase III clinical trials. Ann Oncol. 2014 Dec;25(12):2425–32. DOI: 10.1093/annonc/mdu460
  81. Eilber FC, Brennan MF, Eilber FR, Eckardt JJ, Grobmyer SR, Riedel E, et al. Chemotherapy Is Associated With Improved Survival in Adult Patients With Primary Extremity Synovial Sarcoma. Ann Surg. 2007 Jul;246(1):105–13. DOI: 10.1097/01.sla.0000262787.88639.2b
  82. Woll PJ, Reichardt P, Le Cesne A, Bonvalot S, Azzarelli A, Hoekstra HJ, et al. Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicentre randomised controlled trial. Lancet Oncol. 2012 Oct;13(10):1045–54. DOI: 10.1016/S1470-2045(12)70346-7
  83. Pasquali S, Palmerini E, Quagliuolo V, Martin-Broto J, Lopez-Pousa A, Grignani G, et al. Neoadjuvant chemotherapy in high-risk soft tissue sarcomas: A Sarculator-based risk stratification analysis of the ISG?STS 1001 randomized trial. Cancer. 2022 Jan;128(1):85–93. DOI: 10.1002/cncr.33895
  84. Fiore M, Sambri A, Spinnato P, Zucchini R, Giannini C, Caldari E, et al. The Biology of Synovial Sarcoma: State-of-the-Art and Future Perspectives. Curr Treat Options Oncol. 2021 Dec;22(12):109. DOI: 10.1007/s11864-021-00914-4
  85. Ge YC, Min LM, Liu Q, Wang XL, Wang SF, Chen J, et al. Neo-adjuvant radiation and intratumoral immunotherapy followed by surgery- NARIS trial for extremity soft tissue sarcoma. Future Oncol. 2024 Sep 22;20(30):2233–40. DOI: 10.1080/14796694.2024.2385291
  86. Roulleaux Dugage M, Nassif EF, Italiano A, Bahleda R. Improving Immunotherapy Efficacy in Soft-Tissue Sarcomas: A Biomarker Driven and Histotype Tailored Review. Front Immunol. 2021 Dec 3;12:775761. DOI: 10.3389/fimmu.2021.775761
  87. Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, et al. Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial. Lancet Oncol. 2017 Nov;18(11):1493–501. DOI: 10.1016/S1470-2045(17)30624-1
  88. D’Angelo SP, Araujo DM, Abdul Razak AR, Agulnik M, Attia S, Blay JY, et al. Afamitresgene autoleucel for advanced synovial sarcoma and myxoid round cell liposarcoma (SPEARHEAD-1): an international, open-label, phase 2 trial. The Lancet. 2024 Apr;403(10435):1460–71. DOI: 10.1016/S0140-6736(24)00319-2
  89. Aytekin MN, Öztürk R, Amer K, Yapar A. Epidemiology, incidence, and survival of synovial sarcoma subtypes: SEER database analysis. J Orthop Surg. 2020 May 1;28(2):2309499020936009. DOI: 10.1177/2309499020936009
  90. Vlenterie M, Ho VKY, Kaal SEJ, Vlenterie R, Haas R, Van Der Graaf WTA. Age as an independent prognostic factor for survival of localised synovial sarcoma patients. Br J Cancer. 2015 Dec;113(11):1602–6. DOI: 10.1038/bjc.2015.375
  91. Sultan I, Rodriguez?Galindo C, Saab R, Yasir S, Casanova M, Ferrari A. Comparing children and adults with synovial sarcoma in the Surveillance, Epidemiology, and End Results program, 1983 to 2005: An analysis of 1268 patients. Cancer. 2009 Aug;115(15):3537–47. DOI: 10.1002/cncr.24424
  92. Smolle MA, Parry M, Jeys L, Abudu S, Grimer R. Synovial sarcoma: Do children do better? Eur J Surg Oncol. 2019 Feb;45(2):254–60. DOI: 10.1016/j.ejso.2018.07.006
  93. Yoon SS. The New American Joint Commission on Cancer Staging System for Soft Tissue Sarcomas: Splitting versus Lumping. Ann Surg Oncol. 2018 May;25(5):1101–2. DOI: 10.1245/s10434-018-6419-4
  94. Kang S, Han I, Lee Sa, Kim W, Kim Hs. Clinicopathological characteristics and prognostic factors of T1 (?5 cm) soft tissue sarcoma – A comparative study with T2 (>5 cm) soft tissue sarcoma. Eur J Surg Oncol EJSO. 2014 Apr;40(4):406–11. DOI: 10.1016/j.ejso.2013.12.010
  95. Farkas A, Lirette ST, Al Hmada Y, Collier AB, Barr J, Vijayakumar S, et al. Single-Institution Experience of Synovial Sarcoma. South Med J. 2020 Jan;113(1):16–9. DOI: 10.14423/SMJ.0000000000001054
  96. Ten Heuvel SE, Hoekstra HJ, Bastiaannet E, Suurmeijer AJH. The Classic Prognostic Factors Tumor Stage, Tumor Size, and Tumor Grade are the Strongest Predictors of Outcome in Synovial Sarcoma: No Role for SSX Fusion Type or Ezrin Expression. Appl Immunohistochem Mol Morphol. 2009 May;17(3):189–95.
  97. Guadagnolo BA, Zagars GK, Ballo MT, Patel SR, Lewis VO, Pisters PWT, et al. Long-Term Outcomes for Synovial Sarcoma Treated With Conservation Surgery and Radiotherapy. Int J Radiat Oncol. 2007 Nov;69(4):1173–80. DOI: 10.1016/j.ijrobp.2007.04.056
  98. Fice M, Almajnooni A, Gusho C, Chapman R, Mallikarjunappa S, Batus M, et al. Does synovial sarcoma grade predict oncologic outcomes, and does a low?grade variant exist? J Surg Oncol. 2022 Jun;125(8):1301–11. DOI: 10.1002/jso.26838
  99. Trassard M, Le Doussal V, Hacène K, Terrier P, Ranchère D, Guillou L, et al. Prognostic Factors in Localized Primary Synovial Sarcoma: A Multicenter Study of 128 Adult Patients. J Clin Oncol. 2001 Jan 15;19(2):525–34. DOI: 10.1200/JCO.2001.19.2.525
  100. Sacchetti F, Alsina AC, Muratori F, Scoccianti G, Neri E, Kaya H, et al. Tumor size and surgical margins are important prognostic factors of synovial sarcoma - A retrospective study. J Orthop. 2023 Aug;42:74–9. DOI: 10.1016/j.jor.2023.07.002
  101. Pradhan A, Cheung YC, Grimer RJ, Peake D, Al-Muderis OA, Thomas JM, et al. Soft-tissue sarcomas of the hand: ONCOLOGICAL OUTCOME AND PROGNOSTIC FACTORS. J Bone Joint Surg Br. 2008 Feb;90-B(2):209–14. DOI: 10.1302/0301-620X.90B2.19601
  102. Trovik CS, Bauer HCF, Alvegård TA, Anderson H, Blomqvist C, Berlin Ö, et al. Surgical margins, local recurrence and metastasis in soft tissue sarcomas. Eur J Cancer. 2000 Apr;36(6):710–6. DOI: 10.1016/s0959-8049(99)00287-7
  103. Fanfan D, Alvarez JC, Gonzalez MR, Larios F, Shae J, Pretell-Mazzini J. Foot and Ankle Soft Tissue Sarcomas–Treatment and Oncologic Outcomes: A Systematic Review of the Literature. Foot Ankle Int. 2023 Nov;44(11):1199–207. DOI: 10.1177/10711007231198516
  104. Kainhofer V, Smolle MA, Szkandera J, Liegl-Atzwanger B, Maurer-Ertl W, Gerger A, et al. The width of resection margins influences local recurrence in soft tissue sarcoma patients. Eur J Surg Oncol EJSO. 2016 Jun;42(6):899–906. DOI: 10.1016/j.ejso.2016.03.026


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