Survival and factors affecting the outcome of synovial sarcoma in children and adolescents single institute experience

Abstract

Introduction: To check the impact of the clinical and pathologic features at diagnosis and treatment given, on the outcome of synovial sarcoma in children and adolescents.
Methods: Retrospective analysis of patients below 18 years old diagnosed by synovial sarcoma and treated at Children Cancer
Hospital Egypt 57,357 (CCHE) between July 2007 and December 2016. We reviewed Clinical characteristics, pathological information, treatment modalities and survival data.
Results: We included thirty one patients with median age at diagnosis was 14.8 years. Initial surgical excision was possible in 10 patients (58.8%) while 5 (29.4%) patients underwent surgical excision after response to preoperative chemotherapy. Two patients had unresectable tumor, showed no response to chemotherapy and received radiotherapy as the only local control therapy. Adjuvant radiotherapy only was given in 2 patients and 5 patients received chemotherapy without local radiotherapy and 10 patients received both modalities. The estimated 5-year overall survival and failure free survival rates for the entire group were 84.4% ± 7.2% and 63.8% ± 9.7% respectively, the 5-year OS and FFS were studied in correlation to age of patient, tumor size, different pathological types, site of the lesion and methods of local control. In patients with tumor size below 5 cm when compared to size more than 5 cm with OS was (100%) in comparison to (79.3 ± 9.3) who underwent complete surgical excision either initial or post chemotherapy as it was 66.7% versus 55.6% for those with gross or microscopic residual (p-value = .38). Also, the 3-year failure free survival was 75% versus 56.3% for those smaller than or equal 5 cm and those larger than 5 cm
respectively (p-value = .3).
Conclusion: Increasing number of patients to increase sample size to assess tumor size and complete surgical excision as important prognostic factors as in our study, we found them statistically insignificant. Preoperative chemotherapy can help for delayed excision in patients presented initially with unresectable tumors