Dohrmann G J, Farwell J R, Flannery J T
J Neurosurg. 1976 Sep;45(3):273-83. doi: 10.3171/jns.1976.45.3.0273.
The authors analyze histologically verified cases of ependymoma and ependymoblastoma (malignant ependymoma) occurring in children in Connecticut from 1935 to 1973. Of the 488 central nervous system tumors diagnosed in that period, 44 (9%) of the 467 intracranial neoplasms and five (24%) of the 21 intraspinal tumors were of ependymal origin. An increase in the incidence of ependymomas was noted since the mid-1950's. The mean ages at diagnosis of ependymomas and ependymoblastomas were 5.6 and 5.0 years respectively. The male to female ratio was 0.6:1 for ependymomas and 1.7:1 for ependymoblastomas. Epencymomas were found above and below the tentorium with similar frequency; however, viturally all of the epencymoblastomas occurred supratentorially. Presenting symptoms and physical findings were reviewed. A significant difference (p less than 0.05) was noted in the seizure rates of supratentorial ependymomas (9%) and ependymoblastomas (38%). A significantly increased survival (p less than 0.05) was associated with supratentorial ependymal neoplasms relative to infratentorial from 42 months following diagnosis onward. Contrary tp the reports of no clinical difference between ependymomas and ependymoblastomas, children with supratentorial ependymomas were noted to have a significantly longer survival (p less than 0.05) than those with similarly situated ependymoblastomas, with the difference noted from 18 months following diagnosis onward. The children treated by operation and irradiation had a significantly greater survival (p less than 0.05) than those treated by other methods; furthermore, with this treatment, longer survivals were noted in the ependymoma patients as compared to those with epencymoblastomas. This difference became significant (p less than 0.05) at 27 months after diagnosis. Operative mortality decreased from 40% to 17% in the last decade of the study as compared to the previous decade. Steroid therapy may have contributed to this decreased operative mortality, but it had no statistically significant effect on length of survival. The clinical course of intracranial ependymal neoplasms in adults and children was compared and appeared to be essentially the same.
作者分析了1935年至1973年康涅狄格州儿童中经组织学证实的室管膜瘤和室管膜母细胞瘤(恶性室管膜瘤)病例。在该时期诊断的488例中枢神经系统肿瘤中,467例颅内肿瘤中有44例(9%),21例脊髓肿瘤中有5例(24%)起源于室管膜。自20世纪50年代中期以来,室管膜瘤的发病率有所上升。室管膜瘤和室管膜母细胞瘤诊断时的平均年龄分别为5.6岁和5.0岁。室管膜瘤的男女比例为0.6:1,室管膜母细胞瘤为1.7:1。幕上和幕下室管膜瘤的发现频率相似;然而,几乎所有室管膜母细胞瘤都发生在幕上。回顾了临床表现和体格检查结果。幕上室管膜瘤(9%)和室管膜母细胞瘤(38%)的癫痫发作率存在显著差异(p<0.05)。相对于幕下室管膜肿瘤,幕上室管膜肿瘤自诊断后42个月起生存率显著提高(p<0.05)。与关于室管膜瘤和室管膜母细胞瘤无临床差异的报道相反,幕上室管膜瘤患儿的生存率明显长于幕上位置相似的室管膜母细胞瘤患儿(p<0.05),这种差异自诊断后18个月起就已显现。接受手术和放疗的患儿生存率显著高于接受其他治疗方法的患儿(p<0.05);此外,采用这种治疗方法,室管膜瘤患者的生存期比室管膜母细胞瘤患者更长。这种差异在诊断后27个月时变得显著(p<0.05)。与前十年相比,在研究的最后十年中手术死亡率从40%降至17%。类固醇治疗可能有助于降低手术死亡率,但对生存期无统计学显著影响。比较了成人和儿童颅内室管膜肿瘤的临床病程,结果显示基本相同。