INTRAMEDULLARY SPINAL-CORD TUMORS IN CHILDREN UNDER THE AGE OF 3 YEARS

Over a 13-year period extending from 1980 to 1993, 27 children less th an 3 years of age underwent operation for removal of an intramedullary spinal cord tumor (IMSCT). The majority (18 of 27) of children had un dergone surgery before being referred to New York University (NYU) Med ical Center. The most common reasons for radiological investigation we re pain (42%), motor regression (36%), gait abnormalities (27%), torti collis (27%), and progressive kyphoscoliosis (24%). Forty procedures w ere performed in 27 children. Nine children underwent two operations a nd two children underwent three procedures. A gross-total resection wa s 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 findi ngs: 20 patients' conditions remained the same, five patients improved , and two patients deteriorated. The functional outcomes of a second o peration (NYU-2) were similar. The majority of the children (24 of 27, 89%) had histologically determined low-grade lesions. There were 12 p atients with low-grade astrocytomas (Grades I-III), eight with ganglio gliomas, 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 glioblast oma multiforme. In a median follow-up review of 76 months, two patient s died and two patient were lost to follow up. The 3- and 5-year progr ession-free survival (PFS) rates were 81.7% (standard error of the mea n (SEM) 0.083) and 76.2% (SEM 0.094), respectively. Eight of 24 patien ts suffered a recurrence within a mean time of 45.4 +/- 28.9 months. A ll were treated with surgery (NYU-2). Lesions recurred in three of 12 children with low-grade astrocytomas, two of eight children with gangl iogliomas, 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 schoo l system. The authors conclude that surgery for the removal of IMSCTs in children less than 3 years of age ca be performed radically and saf ely. The postoperative functional performance is determined by the deg ree of the preoperative deficit. It is, therefore, of utmost importanc e to diagnose and treat these children as early as possible. Spinal co rd tumors should be recognized as potentially excisable lesions on the ir initial presentation and when they recur. The optimum treatment for malignant lesions is still to be determined.