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Synovial sarcoma — Clinical Guidelines

Overview

Synovial sarcoma is a rare soft tissue sarcoma often arising in the lower extremities, particularly near joints, but can occur in various locations including the mediastinum and kidney. It typically presents as a slow-growing mass and can mimic other inflammatory or neoplastic conditions. 2 3 4 8

Diagnosis

Clinical Presentation: Slow-growing mass, often in paraarticular regions of extremities. 2 6

Imaging: Ultrasound, MRI, and CT scans to assess extent and location. 2

Biopsy: Ultrasound-guided synovial biopsy recommended for definitive diagnosis. 2

Molecular Testing: Detection of t(X;18) translocation (SYT-SSX fusion) via RT-PCR in paraffin-embedded tissues is crucial for diagnosis, especially in challenging cases. 9

Differential Diagnosis: Includes complex regional pain syndrome, gouty arthritis, and other soft tissue tumors. 3 6

Management

Primary Treatment: Radical surgical excision is considered the treatment of choice. 4

Adjuvant Therapy: Limited efficacy of chemotherapy and radiotherapy; specific drug classes and doses not detailed in abstracts. 4

Monitoring: Regular imaging follow-up post-surgery to monitor for recurrence. 2

Palliative Care: Essential for managing symptoms and quality of life, especially in metastatic cases. 1

Special Populations

Pediatrics: Unique considerations in adolescents, including psychosocial support for gender identity issues alongside cancer treatment. 1

Radiation History: Increased risk of synovial sarcoma in patients with prior radiation therapy to the head and neck region. 13

Key Recommendations

Utilize ultrasound-guided synovial biopsy for accurate diagnosis of synovial sarcoma. (Evidence: Moderate) 2

Confirm synovial sarcoma diagnosis through molecular detection of SYT-SSX fusion transcripts in paraffin-embedded tissues. (Evidence: Strong) 9

Prioritize radical surgical excision as the primary treatment modality. (Evidence: Moderate) 4

Integrate comprehensive palliative care, addressing both physical and psychosocial needs, particularly in pediatric and transgender patients. (Evidence: Expert opinion) 1

References

1 Seachrist K, Somers E, Petras L, Didden E, Deci SE, Tomboc P et al.. Pediatric Palliative Care of a Transgender Adolescent. Pediatrics 2024. link 2 Sitt JC, Griffith JF, Lai FM, Hui M, Chiu KH, Lee RK et al.. Ultrasound-guided synovial Tru-cut biopsy: indications, technique, and outcome in 111 cases. European radiology 2017. link 3 Bernard J, Johnston L, Brennan T. Underlying synovial sarcoma in a patient with a history of complex regional pain syndrome: a case report. The Journal of foot and ankle surgery : official publication of the American College of Foot and Ankle Surgeons 2013. link 4 Loggos S, Kondrafouris K, Oikonomopoulos G, Mitropoulos F. Large monophasic synovial sarcoma of the mediastinum in a 15-year old boy. Interactive cardiovascular and thoracic surgery 2012. link 5 Melese T, G/Hiwot Y, Schneider J. Synovial sarcoma. Ethiopian medical journal 2008. link 6 Du Y, Pullman-Mooar S, Schumacher HR. Synovial sarcoma of the foot mimicking acute gouty arthritis. The Journal of rheumatology 2005. link 7 Rosa N, Samuel N, Lopez-Guerrero, Antonio J, Carmen C, Antonio P et al.. A poorly differentiated synovial sarcoma (SYT/SSX1) expresses neuroectodermal markers: a xenografts and in vitro culture study. Diagnostic molecular pathology : the American journal of surgical pathology, part B 2004. link 8 Törnkvist M, Wejde J, Ahlén J, Brodin B, Larsson O. A novel case of synovial sarcoma of the kidney: impact of SS18/SSX analysis of renal hemangiopericytoma-like tumors. Diagnostic molecular pathology : the American journal of surgical pathology, part B 2004. link 9 Guillou L, Coindre J, Gallagher G, Terrier P, Gebhard S, de Saint Aubain Somerhausen N et al.. Detection of the synovial sarcoma translocation t(X;18) (SYT;SSX) in paraffin-embedded tissues using reverse transcriptase-polymerase chain reaction: a reliable and powerful diagnostic tool for pathologists. A molecular analysis of 221 mesenchymal tumors fixed in different fixatives. Human pathology 2001. link 10 Fisher C. Synovial sarcoma: ultrastructural and immunohistochemical features of epithelial differentiation in monophasic and biphasic tumors. Human pathology 1986. link80083-1) 11 Frassica FJ, Combs JJ, Sim FH. Synovial proliferative disorders: differential diagnosis. Arthroscopy : the journal of arthroscopic & related surgery : official publication of the Arthroscopy Association of North America and the International Arthroscopy Association 1985. link80009-8) 12 Lote K, Dahl O, Vigander T. Pneumothorax during combination chemotherapy. Cancer 1981. link47:7<1743::aid-cncr2820470703>3.0.co;2-g) 13 Mischler NE, Chuprevich T, Tormey DC, Ramirez G, Mack EA. Synovial sarcoma of the neck associated with previous head and neck radiation therapy. Archives of otolaryngology (Chicago, Ill. : 1960) 1978. link

Synovial sarcoma