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Abstract
Purpose: To describe the overall survival, event free survival, clinical features response to therapy and outcome of pediatric Non-Rhabdomyosarcoma soft tissue sarcomas (NRSTS). Results: Synovial sarcoma and fibromatosis were the most frequent histotypes. Median age at diagnosis was 11 years, Tumors were greater than 5 cm in 52 (79 %), high grade in 34 (51.5 %), arose from extremity in 42 (63.6 %) and metastatic at presentation in 14 (21 %) patients. Overall survival at 5-year was 59.3 % for whole series of patients, 67.8 % in patients who underwent complete resection, 63.6 % in patients who had marginal resection, 31.4 % in un-resected patients, and 20.8 % in patients with metastases at onset. Event free survival at 2-year was 17 %. Outcome was unsatisfactory in patients with high-grade tumors, non-extremity location of primary tumor, high-risk stratification and un-resected primary tumor. The rate of response to neo-adjuvant chemotherapy was 28 %. Recurrence rates were greatest in tumors with size > 5cm, positive microscopic margins in high-grade tumors and in patients who did not receive adjuvant radiotherapy in high-grade tumors. Conclusion: The identification of prognostic variables should enable risk-adapted therapies to be planned. Wide surgical resection of primary tumor with negative margins continues to the ministry of therapy. Radiotherapy and adjuvant chemotherapy should reserved for high-grade tumors, inadequate margins and those in whom complete resection can be facilitated by these modalities. Local control was improved by the addition of postoperative RT to high-grade positive margins.