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Benign intradural extramedullary masses in children of northern India
1Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. rajkumar@sgpgi.ac.in
Insights
This study analyzed benign spinal tumors in children, finding nerve sheath tumors are most common. Many children present with severe neurological deficits, highlighting the need for early diagnosis and treatment.
Area of Science:
- Pediatric Neurosurgery
- Oncology
- Spinal Surgery
Background:
- Benign intradural extramedullary tumors in children are rare.
- Understanding their histopathology and clinical presentation is crucial for effective management.
Purpose of the Study:
- To analyze the frequency of different histotypes of benign intradural extramedullary tumors in pediatric patients.
- To evaluate clinical presentation, duration of symptoms, and surgical outcomes.
- To compare findings with those reported in Western literature.
Main Methods:
- Retrospective analysis of 25 pediatric cases with benign intradural extramedullary tumors/lesions.
- Evaluation of symptom duration, clinical profile, and surgical outcomes.
- Use of McCormick functional grading for assessing functional outcomes.
Main Results:
- Nerve sheath tumors (neurofibromas, neurilemomas, schwannomas) constituted approximately 48% of cases.
- Developmental cystic dysraphic lesions were more frequent (28%) than previously reported.
- 68% of children presented with severe neurological compromise (functional grade IV or V).
- All patients, except one with schwannoma, showed functional improvement post-surgery.
Conclusions:
- Nerve sheath tumors are the predominant benign intradural extramedullary tumors in this pediatric cohort.
- Developmental cystic lesions are notably frequent, differing from Western data.
- Delayed presentation with severe neurological deficits is common, emphasizing diagnostic and educational challenges in developing regions.
Abstract:
Twenty-five children with benign intradural extramedullary tumors/lesions were retrospectively analyzed to see the frequency of different histotypes in this location and their variation from the Western world. The duration of symptoms, clinical profile and surgical outcome of these cases were evaluated. The age of children ranged from 1.5 to 18 years, with a mean age of 7.5 years. Thirteen amongst them were male. Follow-up ranged from 3 months to 5 years with a mean of 19 months. McCormick functional grading was used retrospectively to find out the functional outcome in these children. Sixty-eight percent of these cases presented with functional grade IV or V. The mean duration from the appearance of the first symptoms to admission to our hospital in these children was 13 months. Improvement in the functional grade following surgical excision was noted amongst all, except one, who presented with schwannoma, and did not improve from grade III even after a 14-month follow-up. Reoperation/two-staged operation was required in 3 children. The following conclusions were drawn from this study: (1) approximately half (48%) of intradural extramedullary benign tumors are nerve sheath tumors, i.e. neurofibromas, neurilemomas and schwannomas. Giant neurofibromas with giant extraforaminal extension may rarely manifest with a large extraspinal mass; (2) meningioma is an infrequent tumor in this location; (3) developmental cystic dysraphic lesions (neurenteric cyst, arachnoid cysts), unassociated with the stigmata of spinal dysraphism, are significantly more frequent (28%) than reported in the literature; (4) the possibility of infective lesions (granuloma, abscess) causing compressive features cannot be denied even in intradural extramedullary tumors; (5) long-standing lesions, with a long segment involvement, also have a good outcome following a good microsurgical excision, and (6) more than half of the children (68%) present with a severe neurological compromise, either because of ignorance and poor education or due to the scanty diagnostic facilities available at the peripheral hospitals of the developing milieu.
