Synovial sarcoma: A retrospective analyis of 250 patients treated in a single institution

Palmerini, Emanuela; Staals, Eric L.; Zanella, Licciana; Gamberi, Gabriella; Pazzaglia, Laura; Ferrari, C.; Longhi, Alessandra; Alberghini, Marco; Mercuri, Mario; Ferrari, S.

doi:10.1200/jco.2008.26.15_suppl.10506

Cited by 44 publications

(79 citation statements)

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“…The current standard treatment for SS is wide resection followed by polychemotherapy with or without irradiation [11,[19][20][21]. While radiotherapy has a role to play in improving local control of the tumor and, subsequently, overall survival [22], the role of chemotherapy in the treatment of SS remains controversial [7,23]. Due to the rarity of SS, there have been no randomized trials studying the use of chemotherapy in the treatment of SS.…”

Section: Introductionmentioning

confidence: 99%

Synovial sarcoma in patients under 20 years of age: A multicenter study with a minimum follow-up of 10 years

Speth

Krieg

Kaelin

et al. 2011

Journal of Children's Orthopaedics

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Purpose Synovial sarcoma (SS) is an aggressive softtissue tumor noted for late local recurrence and metastasis. This study investigates the long-term outcome of SS in patients of pediatric age and evaluates potential prognostic factors for SS. Methods We performed a retrospective review of 13 SS cases in patients younger than 20 years at the time of diagnosis who had a minimum follow-up of 10 years. The mean follow-up for living patients (n = 8) was 20.1 years (12.1-27.6) and for nonsurvivors (n = 5) 4.9 years (range: 2.6-9.3). Nine patients had unplanned excisions (69%), of which 6 (67%) were performed prior to their referral. Re-excisions were necessary in all 13 patients. The factors sex, tumor site, tumor size, tumor grade, histological subtype, fusion type, and type of treatment were evaluated for their prognostic value. Results Only 2 patients (15%) met the criteria of adequate tumor treatment. Overall, the 5-and 10-year survival rates were 77 and 61%, respectively. The mean time until a local recurrence (n = 5) was 3.2 years (range: 0.7-10.2), while there was a mean time of 2.1 years until the occurrence of late metastases (n = 5; range: 0.8-4.8). A high tumor grade and having a tumor in the trunk were adverse factors in terms of overall, local recurrence-free, and metastasis-free survival. Patients with wide resections or amputations had fewer local recurrences than patients with marginal or intralesional resections. Conclusion Inadequate primary excision of SS results in incomplete excision in the majority of cases. The tumor site, size, and histological grade should be considered when determining a risk-adapted treatment for SS, and wide surgical excision is the surgical intervention of choice. While local recurrence and late metastases appear to occur after a shorter time period in pediatric patients than in adults, in view of the tendency for late recurrence and metastasis with SS, follow-up should be at least 10 years.

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Section: Introductionmentioning

confidence: 99%

Synovial sarcoma in patients under 20 years of age: A multicenter study with a minimum follow-up of 10 years

Speth

Krieg

Kaelin

et al. 2011

Journal of Children's Orthopaedics

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“…10 The clinical presentation of both tumors, namely a large soft tissue mass with a predilection for the extremities of young-to-middle-aged adults, is also quite similar. 5,[11][12][13] The existence of intraneural examples of synovial sarcoma blurs the clinical distinction further. 14 Therefore, the clinical history of NF1 is often the 'gold standard' to establish the diagnosis of malignant peripheral nerve sheath tumor.…”

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confidence: 99%

Diagnostic utility of SOX10 to distinguish malignant peripheral nerve sheath tumor from synovial sarcoma, including intraneural synovial sarcoma

et al. 2014

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Synovial sarcoma and malignant peripheral nerve sheath tumor pose a significant diagnostic challenge given similar histomorphology. The distinction is further complicated by similar immunophenotype and especially by occasional synovial sarcomas that present as intraneural tumors. Although the presence of a t(X;18) rearrangement or expression of TLE1 can help confirm the diagnosis of synovial sarcoma, negative results for these tests are not diagnostic of malignant peripheral nerve sheath tumor. The SOX10 transcription factor, a putative marker of neural crest differentiation, may have diagnostic utility in this differential, but immunohistochemical data are limited. The goal of the present study was to determine the diagnostic utility of SOX10 to discriminate between synovial sarcoma and malignant peripheral nerve sheath tumor. Forty-eight cases of malignant peripheral nerve sheath tumor, all from patients with documented neurofibromatosis, and 97 cases of genetically confirmed synovial sarcoma, including 4 intraneural synovial sarcomas, were immunohistochemically stained for SOX10. The stain was scored for intensity and fraction of cells staining. Thirty-two of 48 malignant peripheral nerve sheath tumors (67%) were SOX10-positive. The majority of malignant peripheral nerve sheath tumors showed Z2 þ staining, but staining did not correlate with grade. By contrast, only 7/97 (7%) synovial sarcomas were SOX10-positive. Only three synovial sarcomas showed Z2 þ staining but, importantly, two of these were intraneural synovial sarcoma. Therefore, SOX10 is a specific (93%), albeit not very sensitive (67%), diagnostic marker to support a diagnosis of malignant peripheral nerve sheath tumor over synovial sarcoma. Furthermore, the stain needs to be interpreted with caution in intraneural tumors in order to avoid a potential diagnostic pitfall. It remains to be determined whether SOX10-positive cells in intraneural synovial sarcoma represent entrapped Schwann cells, synovial sarcoma cells or both.

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“…8 At Bologna's Instituto Ortopedico Rizzoli, however, among patients with localized disease there was no difference in chemotherapy usage between age groups, but children had a better survival than adults. 24 The lack of treatment effect, in particular, chemo therapy, has been confirmed in the Dutch cancer registry study in localized SS, which also demonstrated that younger age is again important for a better outcome, regardless of site or tumor size or use of chemotherapy. 13 Perhaps what we are now clearer about is those patients in whom we may avoid adjuvant chemotherapy, ie, patients with good risk, localized disease.…”

Section: Role Of Chemotherapymentioning

confidence: 87%

Synovial sarcoma: current perspectives

Brennan¹

2016

COAYA

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Synovial sarcoma (SS) is a distinct soft tissue sarcoma, occurring across all ages, from young children to the elderly, but the incidence of SS peaks in young adults. Recently, its biology, specifically the biomarker genomic index, may prove to be the most important prognostic marker, explaining findings such as the positive effect of younger age on outcome. While believed to be a chemosensitive soft tissue sarcoma, surgery remains the most important modality of treatment for many people, especially in localized disease, plus or minus radiotherapy to improve local control. Moving forward, the demonstration that SS has multiple therapeutic targets such as vascular endothelial growth factor, and new emerging targets, allows us to start to consider different systemic therapeutic interventions other than just chemotherapy. This is particularly important for advanced/metastatic SS which, with conventional chemotherapy only, continues to have a very poor outcome. The way forward, therefore, is an all-age trial combining perhaps anti-angiogenesis agents with chemotherapy, focusing on those SS cases whose outcome, as determined by biology, site, or metastatic status, is poor with just conventional sarcoma chemotherapy. This also allows prospective evaluation of the role of genomic index and other biomarkers.

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Synovial sarcoma: A retrospective analyis of 250 patients treated in a single institution

Cited by 44 publications

References 0 publications

Synovial sarcoma in patients under 20 years of age: A multicenter study with a minimum follow-up of 10 years

Synovial sarcoma in patients under 20 years of age: A multicenter study with a minimum follow-up of 10 years

Diagnostic utility of SOX10 to distinguish malignant peripheral nerve sheath tumor from synovial sarcoma, including intraneural synovial sarcoma

Synovial sarcoma: current perspectives

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