Synovial sarcoma: characteristics, challenges, and evolving therapeutic strategies

SS presents challenges in diagnosis and treatment, and there is a need for novel effective therapy for advanced and metastatic disease. Despite international guidelines for the diagnosis and management of SS, there is a risk of diagnostic delay and misdiagnosis following the initial presentation. Early referral of suspected STS to a specialist sarcoma center for biopsy and expert pathology evaluation are recommended by guidelines, such as those published by the ESMO. Surgery at specialist centers is a key pillar of effective management of localized, resectable disease in adults and children with SS, and with the addition of radiotherapy and chemotherapy as indicated by risk assessment tools. In the event of disease progression/metastases, local techniques, such as surgery, radiotherapy (including stereotactic techniques), and thermal ablation for managing individual metastases, as well as novel systemic therapies may also help to extend survival and improve outcomes. The combination of diagnosis, disease evaluation, and multidisciplinary treatment approaches at specialist sarcoma centers can help achieve optimal outcomes for disease management in this patient population.