Akhavan Ali, Binesh Fariba, Shamshiri Hadi, Ghanadi Fazllolah
Department of Radiotherapy, Isfahan University of Medical Sciences, Isfahan, Iran E-mail :
Asian Pac J Cancer Prev. 2014;15(12):4861-4. doi: 10.7314/apjcp.2014.15.12.4861.
The Ewing's sarcoma family is a group of small round cell tumors which accounts for 10-15% of all primary bone neoplasms. The aim of this study was to evaluate the survival of Ewing's sarcoma patients in our province and to determine of influencing factors.
All patients with documented Ewing's sarcoma/ primitive neuroectodermal tumor(PNET) family pathology were enrolled in this study during a period of eight years. For all of them local and systemic therapy were carried out. Overall and event free survival and prognostic factors were evaluated.
Thirty two patients were enrolled in the study. The median age was 17.5 years. Twenty (65.2%) were male and 9 (28.1%) were aged 14 years or less. Mean disease free survival was 26.8 (95%CI; 13.8-39.9) months and five year disease free survival was 26%. Mean overall survival was 38.7 months (95%CI; 25.9-50.6) and median overall survival was 24 months. Five year overall survival was 25%. From the variables evaluated , only presence of metastatic disease at presentation (p value=0. 028) and complete response (p value =0. 006) had significant relations to overall survival.
Survival of Ewing's sarcoma in our province is disappointing. It seems to be mostly due to less effective treatment. Administration of adequate chemotherapy dosage, resection of tumor with negative margins and precise assessment of irradiation volume may prove helpful.
尤因肉瘤家族是一组小圆细胞肿瘤,占所有原发性骨肿瘤的10% - 15%。本研究的目的是评估我省尤因肉瘤患者的生存率,并确定影响因素。
在八年期间,所有有记录的尤因肉瘤/原始神经外胚层肿瘤(PNET)家族病理患者均纳入本研究。对所有患者进行了局部和全身治疗。评估了总生存率、无事件生存率和预后因素。
32例患者纳入研究。中位年龄为17.5岁。20例(65.2%)为男性,9例(28.1%)年龄在14岁及以下。平均无病生存期为26.8个月(95%置信区间;13.8 - 39.9),五年无病生存率为26%。平均总生存期为38.7个月(95%置信区间;25.9 - 50.6),中位总生存期为24个月。五年总生存率为25%。在所评估的变量中,仅就诊时存在转移性疾病(p值 = 0.028)和完全缓解(p值 = 0.006)与总生存率有显著关系。
我省尤因肉瘤的生存率令人失望。这似乎主要是由于治疗效果不佳。给予足够的化疗剂量、切除切缘阴性的肿瘤以及精确评估放疗体积可能会有所帮助。