Intramedullary spinal cord tumors in children under the age of 3 years

被引:144
作者
Constantini, S
Houten, J
Miller, DC
Freed, D
Ozek, MM
Rorke, LB
Allen, JC
Epstein, FJ
机构
[1] NYU,MED CTR,DIV PEDIAT NEUROSURG,NEW YORK,NY 10016
[2] NYU,MED CTR,DIV NEUROPATHOL,NEW YORK,NY 10016
[3] NYU,MED CTR,DIV PEDIAT NEUROONCOL,NEW YORK,NY 10016
[4] NYU,MED CTR,KAPLAN COMPREHENS CANC CTR,NEW YORK,NY 10016
[5] CHILDRENS HOSP PHILADELPHIA,DIV PEDIAT NEUROPATHOL,PHILADELPHIA,PA 19104
关键词
congenital tumor; spinal cord neoplasm; central nervous system neoplasm; magnetic resonance imaging; astrocytoma;
D O I
10.3171/jns.1996.85.6.1036
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Over a 13-year period extending from 1980 to 1993, 27 children less than 3 years of age underwent operation for removal of an intramedullary spinal cord tumor (IMSCT). The majority (18 of 27) of children had undergone surgery before being referred to New York University (NYU) Medical Center. The most common reasons for radiological investigation were pain (42%), motor regression (36%), gait abnormalities (27%), torticollis (27%), and progressive kyphoscoliosis (24%). Forty procedures were performed in 27 children. Nine children underwent two operations and two children underwent three procedures. A gross-total resection was achieved in 72% of the procedures. There was no surgical mortality. A comparison of the preoperative and 3-month postoperative functional grades for the first NYU procedure (NYU-1) yielded the following findings: 20 patients' conditions remained the same, five patients improved, and two patients deteriorated. The functional outcomes of a second operation (NYU-2) were similar. The majority of the children (24 of 27, 89%) had histologically determined low-grade lesions. There were 12 patients with low-grade astrocytomas (Grades I-III), eight with gangliogliomas, two with ganglioglioneurocytomas, one with a glioneurofibroma, and one child with a mixed astro/oligodendroglioma. Two children has anaplastic astrocytomas (Grades II-III) and one child had a glioblastoma multiforme. In a median follow-up review of 76 months, two patients died and two patient were lost to follow up. The 3- and 5-year progression-free survival (PFS) rates were 81.7% (standard error of the mean (SEM) 0.083) and 76.2% (SEM 0.094), respectively. Eight of 24 patients suffered a recurrence within a mean time of 45.4 +/- 28.9 months. All were treated with surgery (NYU-2). Lesions recurred in three of 12 children with low-grade astrocytomas, two of eight children with gangliogliomas, one child with and anaplastic astrocytoma, one child with a ganglioglioneurocytoma, and one child with a glioblastoma multiforme. At follow-up review, most of these children were doing well. Sixteen are in functional Grades I or II and 18 children attend a normal school system. The authors conclude that surgery for the removal of IMSCTs in children less than 3 years of age ca be performed radically and safely. The postoperative functional performance is determined by the degree of the preoperative deficit. It is, therefore, of utmost importance to diagnose and treat these children as early as possible. Spinal cord tumors should be recognized as potentially excisable lesions on their initial presentation and when they recur. The optimum treatment for malignant lesions is still to be determined.
引用
收藏
页码:1036 / 1043
页数:8
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