TY - JOUR
T1 - Treatment at relapse for synovial sarcoma of children and adolescents
T2 - A multi-institutional European retrospective analysis
AU - Ferrari, Andrea
AU - Orbach, Daniel
AU - Bergamaschi, Luca
AU - Schoot, Reineke A.
AU - van Noesel, Max M.
AU - Di Carlo, Daniela
AU - Bisogno, Gianni
AU - Alaggio, Rita
AU - Milano, Giuseppe Maria
AU - Chiaravalli, Stefano
AU - Fuccillo, Fernando
AU - Laurence, Valerie
AU - Corradini, Nadege
AU - Gasparini, Patrizia
AU - Vennarini, Sabina
AU - Pasquali, Sandro
AU - Casanova, Michela
N1 - © 2024 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.
PY - 2024/7
Y1 - 2024/7
N2 - PURPOSE: Though the prognosis for pediatric patients with localised synovial sarcoma (SS) is generally good, the chances of being cured after relapse are limited. This study describes a retrospective multi-institutional series of relapsing SS patients treated at six selected European referral centers for pediatric sarcoma.PATIENTS AND METHODS: The study included 41 patients <21 years with relapsing SS, treated between 2002 and 2022. The analysis included patient's characteristics at first diagnosis, first-line treatments, clinical findings at relapse, and second-line treatment modalities.RESULTS: The first relapse occurred within 3-132 months (median 18 months) after first diagnosis and was local in 34%, metastatic in 54%, and both in 12%. Treatment at first relapse included surgery in 56% of cases, radiotherapy in 34%, and systemic therapy in 88%. In all, 36 patients received second-line medical treatment, that was chemotherapy in 32 cases (with 10 different regimens) and targeted therapy in four. No patient was included in an early-phase clinical trial as second-line therapy-line therapy. Overall response rate was 42%. Median event-free survival (EFS) was 12 months, postrelapse 5-year EFS was 15.8%. Median overall survival (OS) was 30 months, postrelapse 5-year OS was 22.2%. At the Cox's multivariable regression analysis, OS was significantly associated with time and type of relapse.CONCLUSION: Pediatric patients with relapsed SS have a poor prognosis and generally receive an individualized approach, due to the lack of a uniform standardized approach. New comprehensive strategies are needed to improve the knowledge on the biologic landscape of SS and develop tailored prospective clinical trials.
AB - PURPOSE: Though the prognosis for pediatric patients with localised synovial sarcoma (SS) is generally good, the chances of being cured after relapse are limited. This study describes a retrospective multi-institutional series of relapsing SS patients treated at six selected European referral centers for pediatric sarcoma.PATIENTS AND METHODS: The study included 41 patients <21 years with relapsing SS, treated between 2002 and 2022. The analysis included patient's characteristics at first diagnosis, first-line treatments, clinical findings at relapse, and second-line treatment modalities.RESULTS: The first relapse occurred within 3-132 months (median 18 months) after first diagnosis and was local in 34%, metastatic in 54%, and both in 12%. Treatment at first relapse included surgery in 56% of cases, radiotherapy in 34%, and systemic therapy in 88%. In all, 36 patients received second-line medical treatment, that was chemotherapy in 32 cases (with 10 different regimens) and targeted therapy in four. No patient was included in an early-phase clinical trial as second-line therapy-line therapy. Overall response rate was 42%. Median event-free survival (EFS) was 12 months, postrelapse 5-year EFS was 15.8%. Median overall survival (OS) was 30 months, postrelapse 5-year OS was 22.2%. At the Cox's multivariable regression analysis, OS was significantly associated with time and type of relapse.CONCLUSION: Pediatric patients with relapsed SS have a poor prognosis and generally receive an individualized approach, due to the lack of a uniform standardized approach. New comprehensive strategies are needed to improve the knowledge on the biologic landscape of SS and develop tailored prospective clinical trials.
KW - adolescents
KW - children
KW - outcome
KW - prognostic factors relapse
KW - synovial sarcoma
KW - treatment
UR - https://www.scopus.com/pages/publications/85191887793
U2 - 10.1002/pbc.31038
DO - 10.1002/pbc.31038
M3 - Article
C2 - 38679840
AN - SCOPUS:85191887793
SN - 1545-5009
VL - 71
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
IS - 7
M1 - e31038
ER -