Synovial Sarcoma: A Clinical Review

Overview

Journal Curr Oncol

Publisher MDPI

Specialty Oncology

Date 2021 Jun 2

PMID 34069748

Citations 80

Authors

Aaron M Gazendam

Snezana Popovic

Sohaib Munir

Naveen Parasu

David Wilson

Michelle Ghert

Affiliations

Soon will be listed here.

Abstract

Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5-10% of all STS. Synovial sarcoma differs from other STS by the relatively young age at diagnosis and clinical presentation. Synovial sarcomas have unique genomic characteristics and are driven by a pathognomonic t(X;18) chromosomal translocation and subsequent formation of the SS18:SSX fusion oncogenes. Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. Increasing size, age and tumor grade have been demonstrated to be negative predictive factors for both local disease recurrence and metastasis. Wide surgical excision remains the standard of care for definitive treatment with adjuvant radiation utilized for larger and deeper lesions. There remains controversy surrounding the role of chemotherapy in the treatment of SS and there appears to be survival benefit in certain populations. As the understanding of the molecular and immunologic characteristics of SS evolve, several potential systematic therapies have been proposed.

Citing Articles

Heart-breaking tumours: a case series of malignant pericardial effusion.

Wan Muhamad Hatta S, Mirza A, Sunni N, Bashir A Eur Heart J Case Rep. 2025; 9(3):ytaf009.

PMID: 40046975 PMC: 11879449. DOI: 10.1093/ehjcr/ytaf009.

Case report: Primary cardiac synovial sarcoma with suspected connective tissue disease diagnosed by EBUS-TBMB.

Feng Y, Wu C, Chi J, Li L, Wang P, Guo R Front Med (Lausanne). 2025; 12:1515233.

PMID: 39975670 PMC: 11836032. DOI: 10.3389/fmed.2025.1515233.

Synovial sarcoma: A radiologic case report.

Kuruvilla N, Dang B, Dobtsis J Radiol Case Rep. 2025; 20(3):1569-1573.

PMID: 39816460 PMC: 11732846. DOI: 10.1016/j.radcr.2024.11.061.

Primary Pulmonary Synovial Sarcoma With Extensive Myxoid Change Masquerading as a Lung Hydatid Cyst.

Malik S, Ahluwalia C, Ahuja S Ann Thorac Surg Short Rep. 2025; 2(4):669-671.

PMID: 39790583 PMC: 11708504. DOI: 10.1016/j.atssr.2024.06.014.

A case of synovial sarcoma of the right mid-thigh and literature review.

Zhang J, Li Z, Guo G, Jin C, Deng M Oncol Rev. 2025; 18:1445143.

PMID: 39758586 PMC: 11695431. DOI: 10.3389/or.2024.1445143.

References

Kawaguchi N, Ahmed A, Matsumoto S, Manabe J, Matsushita Y . The concept of curative margin in surgery for bone and soft tissue sarcoma. Clin Orthop Relat Res. 2004; (419):165-72. DOI: 10.1097/00003086-200402000-00027. View

Deshmukh R, Mankin H, Singer S . Synovial sarcoma: the importance of size and location for survival. Clin Orthop Relat Res. 2004; (419):155-61. View

Narvani A, Tsiridis E, Saifuddin A, Briggs T, Cannon S . Does image guidance improve accuracy of core needle biopsy in diagnosis of soft tissue tumours?. Acta Orthop Belg. 2009; 75(2):239-44. View

Naing K, Monjazeb A, Li C, Lee L, Yang A, Borys D . Perioperative radiotherapy is associated with improved survival among patients with synovial sarcoma: A SEER analysis. J Surg Oncol. 2014; 111(2):158-64. PMC: 4305005. DOI: 10.1002/jso.23780. View

Michal M, Fanburg-Smith J, Lasota J, Fetsch J, Lichy J, Miettinen M . Minute synovial sarcomas of the hands and feet: a clinicopathologic study of 21 tumors less than 1 cm. Am J Surg Pathol. 2006; 30(6):721-6. DOI: 10.1097/00000478-200606000-00007. View

Bixby S, Hettmer S, Taylor G, Voss S . Synovial sarcoma in children: imaging features and common benign mimics. AJR Am J Roentgenol. 2010; 195(4):1026-32. DOI: 10.2214/AJR.10.4348. View

Wang J, Song Y, Liu X, Jin J, Wang W, Yu Z . Comparison of outcome and toxicity of postoperative intensity-modulated radiation therapy with two-dimensional radiotherapy in patients with soft tissue sarcoma of extremities and trunk. Cancer Med. 2019; 8(3):902-909. PMC: 6434203. DOI: 10.1002/cam4.1919. View

Vlenterie M, Litiere S, Rizzo E, Marreaud S, Judson I, Gelderblom H . 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; 58:62-72. DOI: 10.1016/j.ejca.2016.02.002. View

Jo V, Fletcher C . WHO classification of soft tissue tumours: an update based on the 2013 (4th) edition. Pathology. 2014; 46(2):95-104. DOI: 10.1097/PAT.0000000000000050. View

10.

OSullivan B, Griffin A, Dickie C, Sharpe M, Chung P, Catton C . Phase 2 study of preoperative image-guided intensity-modulated radiation therapy to reduce wound and combined modality morbidities in lower extremity soft tissue sarcoma. Cancer. 2013; 119(10):1878-84. DOI: 10.1002/cncr.27951. View

11.

Aytekin M, Ozturk R, Amer K, Yapar A . Epidemiology, incidence, and survival of synovial sarcoma subtypes: SEER database analysis. J Orthop Surg (Hong Kong). 2020; 28(2):2309499020936009. DOI: 10.1177/2309499020936009. View

12.

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; 44(12):2779-2786. DOI: 10.1007/s00264-020-04708-5. View

13.

Maduekwe U, Hornicek F, Springfield D, Raskin K, Harmon D, Choy E . Role of sentinel lymph node biopsy in the staging of synovial, epithelioid, and clear cell sarcomas. Ann Surg Oncol. 2009; 16(5):1356-63. DOI: 10.1245/s10434-009-0393-9. View

14.

Ferguson P, Griffin A, OSullivan B, Catton C, Davis A, Murji A . Bone invasion in extremity soft-tissue sarcoma: impact on disease outcomes. Cancer. 2006; 106(12):2692-700. DOI: 10.1002/cncr.21949. View

15.

Sleijfer S, Ouali M, Van Glabbeke M, Krarup-Hansen A, Rodenhuis S, Cesne A . Prognostic and predictive factors for outcome to first-line ifosfamide-containing chemotherapy for adult patients with advanced soft tissue sarcomas: an exploratory, retrospective analysis on large series from the European Organization for Research.... Eur J Cancer. 2009; 46(1):72-83. DOI: 10.1016/j.ejca.2009.09.022. View

16.

Eilber F, Brennan M, Eilber F, Eckardt J, Grobmyer S, Riedel E . Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma. Ann Surg. 2007; 246(1):105-13. PMC: 1899195. DOI: 10.1097/01.sla.0000262787.88639.2b. View

17.

Desar I, Fleuren E, van der Graaf W . Systemic Treatment for Adults with Synovial Sarcoma. Curr Treat Options Oncol. 2018; 19(2):13. PMC: 5842271. DOI: 10.1007/s11864-018-0525-1. View

18.

Krieg A, Hefti F, Speth B, Jundt G, Guillou L, Exner U . Synovial sarcomas usually metastasize after >5 years: a multicenter retrospective analysis with minimum follow-up of 10 years for survivors. Ann Oncol. 2010; 22(2):458-67. DOI: 10.1093/annonc/mdq394. View

19.

ENNEKING W . A system of staging musculoskeletal neoplasms. Instr Course Lect. 1988; 37:3-10. View

20.

Guadagnolo B, Zagars G, Ballo M, Patel S, Lewis V, Pisters P . Long-term outcomes for synovial sarcoma treated with conservation surgery and radiotherapy. Int J Radiat Oncol Biol Phys. 2007; 69(4):1173-80. DOI: 10.1016/j.ijrobp.2007.04.056. View