Departments of Medicine and Neurology, Division of Oncology, University of Kentucky, Lexington, KY, USA.
Br J Cancer. 2013 Jun 11;108(11):2367-71. doi: 10.1038/bjc.2013.221. Epub 2013 May 9.
Ependymomas are rare primary gliomas that commonly affect both children and adults, but unique as survival is worse in children.
Data on brain and central nervous system primary malignant and non-malignant ependymal tumours from the Central Brain Tumor Registry of the United States analytic data set and primary malignant ependymal tumours from the SEER 13 registries research data file were used to evaluate incidence and survival, respectively.
The 2004-2009 average annual age-adjusted incidence rate of ependymal tumours was 0.41/100,000. Spinal cord/cauda equina was the primary site at diagnosis for 50-60% of ependymal tumours in adult age groups in contrast to about 20% in children and adolescents. Ependymoma was the most frequent histology in all age groups; however, anaplastic ependymoma comprised about 30% in cases 0-19 years of age compared with about 3-5% in adult age groups. Overall, relative survival was favourable with rates at ∼85% and 75% at 3 and 10 years post diagnosis, respectively. However, children and adolescents, the oldest adult age group, cases diagnosed with anaplastic ependymoma and/or tumour location in a brain site had lowest survival rates.
Paediatric cases had worse outcomes compared with adults for numerous reasons including having a higher percentage of anaplastic ependymomas and greater percentage of cases of intracranial disease.
室管膜瘤是一种罕见的原发性脑胶质瘤，常见于儿童和成人，但儿童的存活率较差。
使用美国中央脑肿瘤登记处的分析数据集和 SEER13 登记处研究数据文件中的脑和中枢神经系统原发性恶性和非恶性室管膜瘤的数据，分别评估发病率和生存率。
2004-2009 年，室管膜瘤的年龄调整年平均发病率为 0.41/100,000。在成年人群中，脊髓/马尾是诊断时的主要部位，占室管膜瘤的 50-60%，而在儿童和青少年中约占 20%。室管膜瘤是所有年龄组中最常见的组织学类型；然而，在 0-19 岁的病例中，间变性室管膜瘤约占 30%，而在成年年龄组中约占 3-5%。总体而言，相对生存率较好，分别为诊断后 3 年和 10 年时约 85%和 75%。然而，儿童和青少年、最年长的成年年龄组、诊断为间变性室管膜瘤和/或肿瘤位于脑部的病例的生存率最低。
由于多种原因，包括间变性室管膜瘤的比例较高和颅内疾病的比例较高，儿科病例的预后比成人差。
