Bordbar Mr, Tasbihi M, Kamfiroozi R, Haghpanah S
Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Amir Oncology Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
Iran J Ped Hematol Oncol. 2014;4(3):89-96. Epub 2014 Jul 20.
Neuroblastoma is the third most common malignancy in children with a very heterogeneous feature. In this study, the epidemiological and clinical characteristics of children with neuroblastoma treated in a referral oncology hospital in Shiraz, Southern Iran, were investigated.
In this historical cohort study, the medical files of 36 children under 18 years old with neuroblastoma were reviewed, who were admitted to Amir Oncology Hospital in Shiraz, Iran from 2006 to 2013. Overall survival and event free survival (EFS) curves were demonstrated by Kaplan Meier methods. Also the effects of demographic and clinical characteristics of the patients on survival were evaluated by Cox regression model.
The median age of diagnosis was 30 months (age range: from 4 to144 months), with M/F ratio of 63.9%. Over 70% of the patients had stage 4 of neuroblastoma at their initial presentations. Adrenal gland comprised 72.2% of the primary tumor site. The most common presenting symptoms were gastrointestinal and constitutional symptoms. The mean overall survival and EFS were 30.75 and 20.56 months, respectively. Among the different variables analyzed, only liver metastasis had an adverse effect on EFS (p=0.025 Hazard ratios 2.83, CI: 1.14-7.02).
This study revealed that the majority of children suffering from neuroblastoma in our center are high stage with disseminated disease at the time of detection. It also warns us about an urgent necessity for holding a re-educational program for general practitioners and pediatricians to review the warning signs of common pediatric cancers such as neuroblastoma.
神经母细胞瘤是儿童中第三常见的恶性肿瘤，具有非常异质性的特征。在本研究中，调查了在伊朗南部设拉子一家转诊肿瘤医院接受治疗的神经母细胞瘤患儿的流行病学和临床特征。
在这项历史性队列研究中，回顾了2006年至2013年期间入住伊朗设拉子阿米尔肿瘤医院的36名18岁以下神经母细胞瘤患儿的病历。采用Kaplan Meier方法绘制总生存曲线和无事件生存（EFS）曲线。还通过Cox回归模型评估了患者的人口统计学和临床特征对生存的影响。
诊断时的中位年龄为30个月（年龄范围：4至144个月），男女比例为63.9%。超过70%的患者在初次就诊时患有4期神经母细胞瘤。肾上腺是原发肿瘤部位的占72.2%。最常见的首发症状是胃肠道和全身症状。平均总生存期和EFS分别为30.75个月和20.56个月。在分析的不同变量中，只有肝转移对EFS有不利影响（p = 0.025，风险比2.83，CI：1.14 - 7.02）。
本研究表明，我们中心大多数患有神经母细胞瘤的儿童在检测时处于晚期且疾病已扩散。它还提醒我们迫切需要为全科医生和儿科医生举办再教育项目，以复习常见儿童癌症如神经母细胞瘤的警示信号。
