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العنوان
Survival Outcome of Soft Tissue Sarcomas,Non-Rhabdomyosarcoma Type in Pediatric Patients Treatedat the National Cancer Institute, Cairo University /
المؤلف
Youssef, Ahmed Mahdy.
هيئة الاعداد
باحث / احمد مهدى يوسف
مشرف / محمد فوزى ابرهيم
مشرف / مجدى ابرهيم الشربينى
مشرف / حسام الدين احمد
الموضوع
Rhabdomyosarcoma.
تاريخ النشر
2015.
عدد الصفحات
170 p. :
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
علم الأورام
تاريخ الإجازة
1/1/2015
مكان الإجازة
جامعة القاهرة - معهد الأورام القومى - طب الاورام الاطفال
الفهرس
Only 14 pages are availabe for public view

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Abstract

To describe the overall survival, event free survival, clinical features response to therapy and outcome of pediatric Non-Rhabdomyosarcoma soft tissue sarcomas (NRSTS). Patients and Methods: We retrospectively reviewed the clinical features and tumor characteristics of all 66 NRSTS patients younger than 18 years who were treated at the National Cancer Institute, Egypt between January 2008 and December 2013. In this subgroup of histotypes, surgery was the mainstay of treatment, chemotherapy was administered to 35 patients, and radiotherapy was administered to 15 patients. 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. Key words: Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcomas, Surgery, Radiation Therapy, Chemotherapy, Prognostic Factors.