Sala F, Talacchi A, Mazza C, Prisco R, Ghimenton C, Bricolo A
Department of Neurologic and Vision Sciences, Section of Neurosurgery, University of Verona, Italy.
Pediatr Neurosurg. 1998 Mar;28(3):135-42. doi: 10.1159/000028637.
Despite several clinical reports on intracranial ependymomas in children, the factors which affect prognosis, and the possibility that certain combinations of factors might limit survival, are still a matter of debate. Between 1976 and 1996 we operated on 35 children with intracranial ependymomas. Postoperative irradiation was given to 27 patients, with associated chemotherapy in 6 cases. Mean follow-up was 62 months. In 12 patients a 5-year follow-up was possible. In October 1996, 18 patients (51.4%) were still alive, the longest disease-free follow-up being 20 years, and the shortest 8 months. We analyzed the prognostic relevance of eight factors. For each factor, different subgroups were distinguished and compared as follows: age at diagnosis (<4 vs. >/=4 years), sex, tumor location (supratentorial vs. infratentorial), tumor size (<4 vs. 4-7 vs. >7 cm), surgical removal (total vs. subtotal), histology (low-grade vs. anaplastic), morphology (solid vs. cystic), adjuvant therapies (treatment vs. no treatment). Two-way contingency tables were made to identify associations between variables. The only significant association was between age and tumor location (p = 0.022): in children under 4, tumors were almost invariably located in the posterior fossa (9 out of 10 cases) with a clear preference for the lateral recess (8 cases). Other correlations were not significant. Kaplan-Meier survival curves were compared to assess the prognostic relevance of each factor. Survival was significantly lower for children under 4, for those with posterior fossa tumors, and for patients with residual tumor (p < 0.05). A multivariate analysis compared variables which significantly affected survival, revealing that age is the most important factor affecting prognosis (p < 0.05), while tumor location and surgical removal do not add any significance to the effect of age on survival. We conclude that age has the strongest prognostic relevance in childhood intracranial ependymomas, while the effect of tumor location on survival may be related to the high incidence of lateral recess ependymomas in younger children.
尽管已有多篇关于儿童颅内室管膜瘤的临床报告，但影响预后的因素以及某些因素组合可能限制生存的可能性仍存在争议。1976年至1996年间，我们为35例儿童颅内室管膜瘤患者实施了手术。27例患者术后接受了放疗，其中6例联合化疗。平均随访时间为62个月。12例患者获得了5年随访。1996年10月，18例患者（51.4%）仍存活，无病生存期最长为20年，最短为8个月。我们分析了8个因素与预后的相关性。对于每个因素，区分并比较了不同亚组，具体如下：诊断时年龄（<4岁与≥4岁）、性别、肿瘤位置（幕上与幕下）、肿瘤大小（<4 cm与4 - 7 cm与>7 cm）、手术切除程度（全切与次全切）、组织学类型（低级别与间变性）、形态（实性与囊性）、辅助治疗（接受治疗与未接受治疗）。制作双向列联表以确定变量之间的关联。唯一显著的关联是年龄与肿瘤位置之间的关联（p = 0.022）：4岁以下儿童的肿瘤几乎都位于后颅窝（10例中有9例），尤其倾向于外侧隐窝（8例）。其他相关性不显著。比较Kaplan - Meier生存曲线以评估每个因素与预后的相关性。4岁以下儿童、后颅窝肿瘤患者以及有残留肿瘤的患者生存率显著较低（p < 0.05）。多因素分析比较了显著影响生存的变量，结果显示年龄是影响预后的最重要因素（p < 0.05），而肿瘤位置和手术切除程度对年龄对生存的影响没有额外的显著作用。我们得出结论，年龄在儿童颅内室管膜瘤的预后相关性中最为显著，而肿瘤位置对生存的影响可能与年幼儿童中外侧隐窝室管膜瘤的高发病率有关。
