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6.P.03 HIGH-RISK SYNOVIAL SARCOMA OF CHILDREN TREATED WITH INTENSIVE CHEMOTHERAPY, SURGERY AND RADIATION THERAPY



Abstract

Synovial sarcoma is the most common NRSTS, that typically affects the extremities of adolescents. To improve the results of the treatment of synovial sarcoma for children and adolescents is the target of this study. 19 children and adolescents at the mean age of 10,84±3,28 years (9 males, 10 females) with synovial sarcoma were treated between 1999 and 2008 years at the Research Institution of Pediatric Oncology in the Russian Cancer Center. Histologically, 5 patients had the biphasic,12 had the monophasic, and 2 of them had the poorly differentiated pattern. The most often affected area was the area of the lower extremity – 10 cases, the area of the upper extremity was affected in 3 cases, and the trunk – 6 cases. According to the staging systems adopted, the size > 5cm (TB) was reported in 12 cases. Five patients (non-staging) had relapse of disease. Four patients had nodal involvement, and 4 had distant metastases (mostly at lungs).

The general scheme of the treatment included: 8 courses of chemotherapy (used ifosfamide or cyclophosphamide, ethoposide, carboplatine); the harvesting and preservation of the stem cells after the stimulation of the haemophoesis by G-CSF, the stage of the local control of the tumor consisting of the surgical ablation of the primary lesion (in 1 case it was not available) and the radiotherapy of the initial tumor and metastasis left after the induction.

The partial effect was registered by most of the patients – 80%. We observed 1 case of progression of the disease during inductive CT. The toxicity of intensive chemotherapy was reduced by support of sub transplantation doses of peripheral blood stem cells – 0,9-1,5±0,1·106 per kg. In our research we have analyzed the 5-year overall and disease free survival. Thus, 5-year disease-free survival was 66,1±11,3 %, overall 5-year survival −75,6±10,6%.

Correspondence should be addressed to Professor Stefan Bielack, Olgahospital, Klinikum Stuttgart, Bismarkstrasse 8, D-70176 Stuttgart, Germany. Email: s.bielack@klinikum_stuttgart.de