BIOLOGY OF SYNOVIAL SARCOMA

Ann Oncol. 2014 Dec 8. pii: mdu562. [Epub ahead of print]

Synovial sarcoma in children and adolescents: the European Pediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005).

Ferrari A1, De Salvo GL2, Brennan B3, van Noesel MM4, De Paoli A2, Casanova M5, Francotte N6, Kelsey A7, Alaggio R8, Oberlin O9, Carli M10, Ben-Arush M11, Bergeron C12, Merks JH13, Jenney M14, Stevens MC15, Bisogno G10, Orbach D16.

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Abstract

BACKGROUND: 

To report the results of the first European prospective nonrandomized trial dedicated to pediatric synovial sarcoma.

PATIENTS AND METHODS: 

From August 2005 to August 2012, 138 patients <21 60="" 9="" a="" abstracttext="" according="" and="" based="" centers="" chemotherapy="" countries="" different="" ifosfamide-doxorubicin="" in="" including="" involvement.="" multimodal="" nodal="" nonmetastatic="" old="" on="" patients="" radiotherapy="" registered="" risk="" sarcoma="" site="" size="" stage="" stratification="" surgical="" synovial="" therapy="" to="" treated="" tumor="" were="" with="" years="">

RESULTS: 

With a median follow-up of 52.1 months (range 13.8-104.4 months), event-free survival (EFS) was 81.9% and 80.7%, and overall survival (OS) was 97.2% and 90.7%, at 3 and 5 years, respectively. The only significant prognostic variable at univariate analysis was the risk group: 3-year EFS was 91.7% for low-risk, 91.2% for intermediate-risk, and 74.4% for high-risk cases. In 24 low-risk patients (completely resected tumor ≤5 cm in size) treated with surgery alone, there were two local relapses and no metastatic recurrences. Among 67 high-risk patients (unresected, or axial tumor or nodal involvement), 66 underwent surgery after neoadjuvant chemotherapy. Response to chemotherapy was 55.2%, including 22.4% cases with complete or major partial remissions, and 32.8% with minor partial remissions.

CONCLUSION: 

This study demonstrates that collaborative prospective studies on rare pediatric sarcomas are feasible even on a European scale, with excellent treatment compliance. The overall results of treatment were satisfactory, with higher survival rates than those previously published by pediatric groups. Nonetheless, larger, international projects are needed, based on a cooperative effort of pediatric and adult oncologists.

CLINICAL TRIALS NUMBER: