Akhavan A, Binesh F, Hashemi A, Shamshiri H
Department Of Radiotherapy,Isfahan University Of Medical Sciences,Isfahan,Iran.
Department Of Pathology,Shahid Sadoughi University Of Medical Sciences,Yazd,Iran.
Iran J Ped Hematol Oncol. 2014;4(3):97-102. Epub 2014 Jul 20.
Ewing's sarcoma family is a group of small round cells tumors. The aim of this study is to evaluate clinicopathologic characteristics and outcome of Ewing's sarcoma in children and adolescents in Yazd, Iran.
All patients under 19 years with documented pathology of Ewing's sarcoma family tumor who referred to Shahid Ramazanzadeh Radiotherapy center between 2002 to 2010 were enrolled in this retrospective study. Overall survival and disease free survival and prognostic factors were evaluated.
Among approximately 80,000 patients who referred to Shahid Sadoughi pathology department, over an 8-year period, the total number of patients with Ewing sarcoma was 32, of which, 18 cases were under the age 19 . The mean age was 13.72 years. Five patients (27.8%) had metastatic disease at the time of diagnosis. Complete response had been achieved in 8 (44.4%) of the patients. Local recurrence occurred in 4 (22.2%) of the patients. During the follow up 13 (72.2%) of the patients showed metastases. The mean overall survival was 34.79 months (95% CI: 22.27-47.32) .One, two, four and five year survival was 72%, 39%, 25% and 17% respectively. Complete remission occurred in 10 patients (63.6%). A trend of better overall survival was found in these patients (p=0. 55). When the brain and bone metastases occurred, the overall survival decreased significantly (p=0. 003 ).
The overall survival rate of Ewing's sarcoma is very low in comparison with other parts of the world.
尤因肉瘤家族是一组小圆细胞肿瘤。本研究旨在评估伊朗亚兹德儿童和青少年尤因肉瘤的临床病理特征及预后。
对2002年至2010年间转诊至沙希德拉马赞扎德放射治疗中心的所有19岁以下经病理证实为尤因肉瘤家族肿瘤的患者进行了这项回顾性研究。评估总生存期、无病生存期及预后因素。
在8年期间转诊至沙希德萨多基病理科的约80000名患者中，尤因肉瘤患者总数为32例，其中18例年龄在19岁以下。平均年龄为13.72岁。5例患者（27.8%）在诊断时已有转移性疾病。8例患者（44.4%）达到完全缓解。4例患者（22.2%）出现局部复发。随访期间，13例患者（72.2%）出现转移。平均总生存期为34.79个月（95%可信区间：22.27 - 47.32）。1年、2年、4年和5年生存率分别为72%、39%、25%和17%。10例患者（63.6%）出现完全缓解。这些患者的总生存期有更好的趋势（p = 0.55）。当发生脑转移和骨转移时，总生存期显著降低（p = 0.003）。
与世界其他地区相比，尤因肉瘤的总生存率非常低。
