Fact-sheet: Synovial sarcoma
Updated on 09/17/2021 at 12:16 PM
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Definition
-malignant mesenchymal tumor (not of synovial origin) accounting for 5 to 10% of soft tissue tumors. 2.5/10,000 inhabitants
-Develops mainly from soft tissues. Slow progression
-Predilection age: children-adolescents and especially young adults
-Most common malignant soft tissue tumor in children after rhabdomyosarcoma
-Predominant location in the extremities (lower limbs+++), frequently juxta-articular in topography but without an intra-articular starting point
-Numerous locations are possible: retropharyngeal, laryngeal, pharyngeal, sellar, pleural, pulmonary, pericardial, retroperitoneal
-Secondary locations: pulmonary, lymph nodes
-55% 5-year survival
Clinical features
The 2 most common presenting symptoms:
–a usually well-defined mass, slow-growing, painful or not
–pain may be isolated or may precede the appearance of a mass by several weeks or even years.
The clinical presentation may mistakenly suggest diagnoses of tendinitis, cysts, post-cyst pain, post-traumatic pain…
For visceral locations, the diagnosis of synovial sarcoma is only established by pathological examination (molecular biology)
CT
-Nonspecific: highly vascularized soft tissue mass, with a few calcifications, heterogeneous peripheral enhancement. Intralesional hemorrhage is frequent.
Nuclear medicine
-PET scan shows FDG accumulation at the tumor site, useful when searching for recurrence
Management
-Treatment: surgical, chemotherapy +/- radiotherapy if resection is incomplete