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儿童颅内室管膜瘤

Intracranial ependymomas in children.

作者信息

Undjian S, Marinov M

机构信息

Department of Neurosurgery, Medical Academy, Sofia, Bulgaria.

出版信息

Childs Nerv Syst. 1990 May;6(3):131-4. doi: 10.1007/BF00308488.

Abstract

During a 20-year period (1964-1983), the authors managed 60 children with intracranial ependymomas. In 37 patients the tumors were located in the posterior cranial fossa, and in the remaining 23 they were supratentorial. The histological examination in 26 children revealed ependymoblastomas. The average duration of the clinical evolution was 4 months and 5 days in the supratentorial neoplasms and 3 months and 6 days in the subtentorial. Besides the intracranial hypertension, present in about 80% of the patients, specific localizing signs were seen in 70% of the children with subtentorial and in 40% of those with supratentorial tumors. Because of the growth pattern in posterior fossa ependymomas and despite the prevalence of histologically benign neoplasms, radical resection was accomplished in only 21.6% of the cases, as compared with 30.4% in supratentorial tumors. The postoperative mortality remains high: 29.7% in subtentorial tumors and 17.4% in supratentorial. The 1-year survival rate in subtentorial neoplasms was 70.3%, the 3-year survival 29.7%, and the 5-year survival 16.2%. The corresponding figures for supratentorial ependymomas more favorable: 82.6%, 43.5%, and 26.1%, respectively. The most important factors for improving the outcome at the present time appear to be as radical a resection as possible, supplemented with postoperative radiotherapy and chemotherapy.

摘要

在20年期间(1964 - 1983年),作者们治疗了60例颅内室管膜瘤患儿。37例患者的肿瘤位于后颅窝,其余23例位于幕上。26例患儿的组织学检查显示为成室管膜细胞瘤。幕上肿瘤的临床病程平均持续时间为4个月零5天,幕下肿瘤为3个月零6天。除了约80%的患者存在颅内高压外,70%的幕下肿瘤患儿和40%的幕上肿瘤患儿出现了特定的定位体征。由于后颅窝室管膜瘤的生长方式,尽管组织学上良性肿瘤占多数,但仅21.6%的病例实现了根治性切除,而幕上肿瘤的这一比例为30.4%。术后死亡率仍然很高:幕下肿瘤为29.7%,幕上肿瘤为17.4%。幕下肿瘤的1年生存率为70.3%,3年生存率为29.7%,5年生存率为16.2%。幕上室管膜瘤的相应数字更乐观,分别为82.6%、43.5%和26.1%。目前改善预后的最重要因素似乎是尽可能进行根治性切除,并辅以术后放疗和化疗。

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