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Intramedullary spinal cord tumors in children under the age of 3 years
S Constantini1, J Houten, D C Miller
1Division of Pediatric Neurosurgery, New York University Medical Center, New York, New York, USA.
Insights
Surgery for pediatric intramedullary spinal cord tumors (IMSCTs) in young children is safe and effective, with most patients maintaining or improving function. Early diagnosis and treatment are crucial for optimal outcomes in these challenging cases.
Area of Science:
- Pediatric Neurosurgery
- Pediatric Oncology
- Spinal Cord Oncology
Background:
- Intramedullary spinal cord tumors (IMSCTs) in children under 3 years old are rare and challenging.
- Symptomatic presentation often includes pain, motor regression, gait abnormalities, torticollis, and progressive kyphoscoliosis.
- Many patients undergo initial surgeries before referral to specialized centers.
Purpose of the Study:
- To evaluate the safety and efficacy of surgical resection for IMSCTs in very young children.
- To analyze functional outcomes, recurrence rates, and survival following surgery.
- To determine factors influencing postoperative functional status.
Main Methods:
- Retrospective review of 27 children under 3 years of age who underwent surgery for IMSCTs between 1980 and 1993.
- Analysis of surgical procedures, including gross-total resection rates and complications.
- Assessment of preoperative and postoperative functional grades, histopathological findings, and long-term follow-up data.
Main Results:
- No surgical mortality was observed.
- Gross-total resection was achieved in 72% of procedures.
- Postoperative functional outcomes showed stability in 20 patients, improvement in five, and deterioration in two after the first surgery; similar results were seen after a second operation.
- The 3- and 5-year progression-free survival rates were 81.7% and 76.2%, respectively.
- 89% of lesions were low-grade; common types included astrocytomas and gangliogliomas.
- Recurrence occurred in 8 of 24 patients, treated successfully with re-operation.
Conclusions:
- Surgical removal of IMSCTs in children under 3 is safe and can be performed radically.
- Postoperative functional outcomes are largely dependent on the preoperative neurological deficit.
- Early diagnosis and prompt treatment are essential for improving outcomes in pediatric IMSCTs.
- Spinal cord tumors should be considered potentially resectable lesions at initial presentation and upon recurrence.
Abstract:
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 had 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 patients 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 an 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 can 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.