[Synovial sarcoma in children and adolescents]

Area of Science:

• Oncology
• Genetics
• Pediatric Medicine

Context:

• Synovial sarcoma (SS) is a rare, high-grade malignant mesenchymal tumor primarily affecting children, adolescents, and young adults.
• Over 90% of SS cases exhibit a characteristic t(X;18)(p11.2;q11.2) translocation, leading to SYT-SSX1 or SYT-SSX2 fusion genes, crucial for diagnosis.
• Pediatric SS typically presents as a localized soft tissue tumor of the extremities with low rates of lymph node or distant metastasis.

Purpose:

• To summarize the characteristics, treatment modalities, and outcomes of synovial sarcoma in pediatric and adolescent populations.
• To highlight the differences in survival rates and therapeutic approaches between children and adolescents with SS.
• To discuss the role of prognostic factors, multimodal therapy, and emerging genomic data in optimizing SS management.

Summary:

• Complete surgical resection is the standard treatment for SS, with varying therapeutic strategies for pediatric and adult patients.
• While clinical and biological features are similar, children with SS demonstrate better 5-year overall survival (84%) compared to adolescents (60%).
• Multimodal therapeutic approaches, informed by prognostic factors and supported by studies like the EpSSG NRSTS 05, show promising 5-year OS rates exceeding 90% in localized SS.

Impact:

• Establishes the effectiveness of multimodal therapy and highlights the need for age-stratified treatment strategies in pediatric and adolescent SS.
• Emphasizes the importance of considering prognostic factors and tumor genomics for personalized treatment planning.
• Underscores the necessity for multinational prospective studies in young patients to advance optimal management of this rare sarcoma.