Schwann cells are generally present in the nerve root, not in the spinal cord. Therefore, intramedullary schwannomas are rare and correct preoperative diagnosis is often difficult. To date, less than 50 cases of intramedullary schwannoma with no sign of neurofibromatosis have been reported in the English literature, of which 5 occurred in children. Here, we report a case of primary thoracic intramedullary schwannoma which occurred at the age of 11, confirmed by intraoperative frozen biopsy. The patient presented with decreased sensation below the T6 dermatome at the right side and right lower extremity weakness with urge incontinence. The ideal treatment for intramedullary schwannoma of histologically benign nature is surgical resection. However, the mass was subtotally resected due to unfavorable intraoperative somatosensory-evoked potential findings, and postoperative fractionated radiotherapy followed. She has been alive with the residual intramedullary mass with no discernible changes during the 11.5 years of follow-up. When neurosurgeons, pediatricians and radiologists encounter an intramedullary tumor in a pediatric patient, intramedullary schwannoma should be included in the differential diagnoses because it might be curable if excised.
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