6.P.12 TREATMENT RESULTS OF PEDIATRIC NON-RHABDOMYOSARCOMA SOFT TISSUE SARCOMAS

Abstract

The nonrhabdomyosarcoma soft tissue sarcomas constitute a heterogenous group of rare mesenchymal tumors that account for less than %5 of pediatric cancers. Their biology and optimal treatment is not well understood. This study retrospectively analyses a small subset of surgically treated patients.

Fifteen patients with a mean age of 11.4 years (4 months – 16 years) were followed-up for a mean of 48 (2–124) months. The histologic diagnosis was synovial sarcoma in 5 patients; soft tissue Ewing Sarcoma in 3; fusiform cell sarcoma in 3; malignant peripheral nerve sheath tumor in 2; fibromyxoid sarcoma in 1 and myxoid liposarcoma in 1. The tumor was located in the upper extremity in 6 patients; thigh in 4; inguinal region in 2, foot in 2 and gluteal region in 1. Eight patients received preoperative chemotherapy and 5 received preoperative radiotherapy. Two patients had pulmonary metastasis at the time of admission. Thirteen patients were operated by limb salvage procedures and 2 underwent amputation.

Tumor resection was radical in 2 patients, wide in 12 and marginal in 1. Five patients received adjuvant chemotherapy and 5 received adjuvant radiotherapy. There were 5 local recurrences after 23.8 (14–40) months; three patients underwent wide resection for recurrence and two received chemotherapy and radiotherapy. Three patients had systemic metastases after a mean of 32.3 (27–40) months. There were no major complications but local wound problems were encountered. Three patients died of disease after a mean of 65.3 months (32–124 months). Two patients had metastatic disease and 10 had no evidence of disease in the last follow-up visit.

Pediatric soft tissue sarcomas can be treated following the principles of adult soft tissue sarcomas, except for wide utilization of radiotherapy in neoadjuvant and adjuvant setting. Treatment results are similar to adult patients.