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成人WHO二级和三级幕上半球室管膜瘤:病例系列及治疗选择综述

WHO Grade II and III supratentorial hemispheric ependymomas in adults: case series and review of treatment options.

作者信息

Niazi Toba N, Jensen Elizabeth M, Jensen Randy L

机构信息

Department of Neurosurgery, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT 84132, USA.

出版信息

J Neurooncol. 2009 Feb;91(3):323-8. doi: 10.1007/s11060-008-9717-z. Epub 2008 Nov 1.

Abstract

Supratentorial ependymomas and their anaplastic variants are relatively uncommon central nervous system neoplasms that afflict both adults and children. Whereas the treatment algorithm in the pediatric population is well established, however, treatment in the adult population is less defined. In our case series of three adult patients with supratentorial ependymomas, two patients had tumors of WHO Grade III (anaplastic variant) and one had tumor of WHO Grade II. In all patients, gross total resection was achieved. Additional radiation therapy was administered in the Grade III patients. Twenty-four-month follow-up in case 1 yielded no tumor recurrence and no requirement of adjuvant chemotherapy. In case 2, tumor recurred with leptomeningeal gliomatosis by 6 months. Addition of platinum-based chemotherapy did not improve long-term survival; the patient succumbed to the disease after 14 months. In case 3 (WHO Grade II), no radiation therapy was required. Tumor did not recur during the 42-month follow-up. In our experience, gross total resection was achieved in all patients with hemispheric supratentorial WHO Grade II or Grade III ependymomas with additional radiation therapy for Grade III variants. All patients require initial close serial imaging and follow-up. The role of chemotherapy is still uncertain but may be necessary in younger patients who may have tumors that behave more like the pediatric ependymomas.

摘要

幕上室管膜瘤及其间变性变体是相对罕见的中枢神经系统肿瘤,可累及成人和儿童。然而,虽然儿科人群的治疗方案已确立,但成人患者的治疗方案仍不太明确。在我们的三例幕上室管膜瘤成年患者病例系列中,两名患者患有世界卫生组织(WHO)III级肿瘤(间变性变体),一名患者患有WHO II级肿瘤。所有患者均实现了肿瘤全切。III级患者接受了额外的放射治疗。病例1随访24个月未出现肿瘤复发,也无需辅助化疗。病例2在6个月时肿瘤复发并伴有软脑膜胶质瘤病。添加铂类化疗并未改善长期生存率;该患者在14个月后死于该疾病。病例3(WHO II级)无需放疗。在42个月的随访期间肿瘤未复发。根据我们的经验,所有半球幕上WHO II级或III级室管膜瘤患者均实现了肿瘤全切,III级变体患者还接受了额外的放射治疗。所有患者均需要进行初始密切的系列影像学检查和随访。化疗的作用仍不确定,但对于可能患有更类似儿科室管膜瘤的肿瘤的年轻患者可能是必要的。

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