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儿童高级别脊髓肿瘤。

High-grade pediatric spinal cord tumors.

作者信息

Merchant T E, Nguyen D, Thompson S J, Reardon D A, Kun L E, Sanford R A

机构信息

Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, Tenn. 38105,

出版信息

Pediatr Neurosurg. 1999 Jan;30(1):1-5. doi: 10.1159/000028751.

Abstract

Our institutional experience with high-grade pediatric spinal cord tumors includes 11 children treated during the period of 1981-1997. All patients underwent a biopsy or an attempt at resection and received postoperative radiation therapy. Three patients had a gross-total resection of their primary tumor, 6 patients had a subtotal resection and the remaining 2 were biopsied. Histologically, these tumors were characterized as anaplastic astrocytoma (n = 6), glioblastoma multiforme (n = 3) or anaplastic oligodendroglioma (n = 2). Three patients were treated with craniospinal irradiation (38-48 Gy) in addition to a boost to the residual tumor. The median dose to the primary site for all patients was 48.6 Gy (range 38-55 Gy). The median overall survival was 13 months (range 8-149 months). Only 2 patients were alive at 138 and 149 months following radiation therapy. The median progression-free survival following radiation therapy was 10 months (range 2-80 months). There was no difference in progression-free or overall survival for those diagnosed with glioblastoma multiforme when compared to patients diagnosed with anaplastic astrocytoma or anaplastic oligodendroglioma. The pattern of failure was either diffuse or local. For the patients who failed diffusely (n = 6), the median progression-free survival was 2 months compared to 23 months for those whose failure was entirely local (p < 0.01). The median overall survival was significantly shorter for those who failed diffusely compared to those who failed locally (10 vs. 37 months, p < 0.01). High-grade spinal cord tumors in children have a poor prognosis based on this report. It is important to document the extent of disease accurately prior to the initiation of radiation therapy, since a subset of these patients progress rapidly outside of the field of irradiation.

摘要

我们机构在治疗儿童高级别脊髓肿瘤方面的经验涵盖了1981年至1997年期间治疗的11名儿童。所有患者均接受了活检或肿瘤切除尝试,并接受了术后放射治疗。3例患者实现了原发肿瘤的全切,6例患者进行了次全切除,其余2例进行了活检。从组织学上看,这些肿瘤的特征为间变性星形细胞瘤(n = 6)、多形性胶质母细胞瘤(n = 3)或间变性少突胶质细胞瘤(n = 2)。3例患者除了对残留肿瘤进行加量照射外,还接受了全脑全脊髓照射(38 - 48 Gy)。所有患者原发部位的中位照射剂量为48.6 Gy(范围38 - 55 Gy)。中位总生存期为13个月(范围8 - 149个月)。放疗后仅2例患者分别在138个月和149个月时存活。放疗后的中位无进展生存期为10个月(范围2 - 80个月)。与诊断为间变性星形细胞瘤或间变性少突胶质细胞瘤的患者相比,诊断为多形性胶质母细胞瘤的患者在无进展生存期或总生存期方面没有差异。失败模式为弥漫性或局部性。对于弥漫性失败的患者(n = 6),中位无进展生存期为2个月,而对于完全局部失败的患者,中位无进展生存期为23个月(p < 0.01)。与局部失败的患者相比,弥漫性失败的患者中位总生存期明显更短(10个月对37个月,p < 0.01)。根据本报告,儿童高级别脊髓肿瘤预后较差。在开始放疗前准确记录疾病范围很重要,因为这些患者中有一部分在照射野之外进展迅速。

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