基于监测、流行病学和最终结果（SEER）数据的儿童脑干原始神经外胚层肿瘤发病率
The incidence of brainstem primitive neuroectodermal tumors of childhood based on SEER data.
作者信息
Chamdine Omar, Elhawary Ghada Ahmad Saad, Alfaar Ahmad Samir, Qaddoumi Ibrahim
机构信息
King Fahad Specialist Hospital, Dammam, Saudi Arabia.
Ain Shams University Hospitals, Cairo, Egypt.
出版信息
Childs Nerv Syst. 2018 Mar;34(3):431-439. doi: 10.1007/s00381-017-3687-4. Epub 2018 Jan 3.
PURPOSE
Incidence of BS primitive neuroectodermal tumors (BS-PNET) in children is not reported to date. Our main objectives were to estimate the incidence and report the outcome of BS-PNET in children.
METHODS
Data were collected using the Surveillance Epidemiology and End Results cancer registry.
RESULTS
From 1973 to 2013, we identified 83 pediatric patients (aged 0-21 years). Patients were divided into two age groups (0-3 years and 4-21 years). Median overall survival was 53 months. Patients in the older age group had a significant survival advantage (P < 0.001), as did those who received three modalities of therapy (surgery, chemotherapy, and radiation therapy) (P < 0.001) and patients with gross or subtotal tumor resection (P < 0.001).
CONCLUSIONS
This study presents the first estimate of incidence and the largest cohort of pediatric BS-PNETs to date. A high index of suspicion of BS-PNET in similar cases is crucial for diagnosis, treatment, and outcome.
目的
儿童脑干原始神经外胚层肿瘤（BS-PNET）的发病率迄今尚无报道。我们的主要目的是估计儿童BS-PNET的发病率并报告其预后。
方法
使用监测、流行病学和最终结果癌症登记处收集数据。
结果
1973年至2013年，我们确定了83例儿科患者（年龄0至21岁）。患者分为两个年龄组（0至3岁和4至21岁）。总生存期中位数为53个月。年龄较大组的患者有显著的生存优势（P<0.001），接受三种治疗方式（手术、化疗和放疗）的患者也是如此（P<0.001），肿瘤大体或次全切除的患者同样如此（P<0.001）。
结论
本研究首次对发病率进行了估计，并呈现了迄今为止最大的儿童BS-PNET队列。在类似病例中对BS-PNET保持高度怀疑指数对诊断、治疗和预后至关重要。
Zotero 插件