Ferrari A, Orbach D, Bergamaschi L, Schoot RA, van Noesel MM, Di Carlo D, Bisogno G, Alaggio R, Milano GM, Chiaravalli S, Fuccillo F, Laurence V, Corradini N, Gasparini P, Vennarini S, Pasquali S, Casanova M.Pediatr Blood Cancer. 2024 Jul;71(7):e31038. doi: 10.1002/pbc.31038. Epub 2024 Apr 28.PMID: 38679840
Synovial sarcoma (SS) is a rare cancer that typically has a good outcome in children and adolescents when it is localized, but curing it after a relapse is challenging. This study looked at 41 young patients who experienced a relapse of SS to understand how they were treated and their outcomes.
The first relapse happened between 3 months and 11 years after the initial diagnosis, with a median of 18 months. For 34% of patients, the relapse was limited to the original site, while 54% had it spread to other areas (metastatic), and 12% had both. Treatment for the first relapse often included surgery (56%), radiotherapy (34%), and systemic treatments like chemotherapy (88%).
Most patients (36) received further treatments with different types of chemotherapy or targeted therapy, but no patients participated in early-phase clinical trials for new therapies at this stage. The overall response to these treatments was 42%, but survival rates were low. Only 16% of patients avoided further progression or relapse for five years, and 22% survived five years after their relapse.
The study highlights the difficulties in treating relapsed SS in young patients. A major finding is the lack of a standardized treatment approach, which leads to individualized care based on the patient's situation. The authors emphasize the urgent need for better understanding of SS biology and for developing new, targeted treatments through clinical trials to improve outcomes for these patients.
The first relapse happened between 3 months and 11 years after the initial diagnosis, with a median of 18 months. For 34% of patients, the relapse was limited to the original site, while 54% had it spread to other areas (metastatic), and 12% had both. Treatment for the first relapse often included surgery (56%), radiotherapy (34%), and systemic treatments like chemotherapy (88%).
Most patients (36) received further treatments with different types of chemotherapy or targeted therapy, but no patients participated in early-phase clinical trials for new therapies at this stage. The overall response to these treatments was 42%, but survival rates were low. Only 16% of patients avoided further progression or relapse for five years, and 22% survived five years after their relapse.
The study highlights the difficulties in treating relapsed SS in young patients. A major finding is the lack of a standardized treatment approach, which leads to individualized care based on the patient's situation. The authors emphasize the urgent need for better understanding of SS biology and for developing new, targeted treatments through clinical trials to improve outcomes for these patients.
