Intraspinal clear-cell meningioma: case report and review of literature.

BACKGROUND: Intraspinal clear-cell meningioma (CCM) is a rare morphological variant of meningioma with only 16 documented cases. We report one case and review the literature regarding intraspinal CCM.

CASE PRESENTATION: A 2-year-old boy and a 2-month-old male infant presented with knee pain and leg weakness. Magnetic resonance imaging revealed an intradural extramedullary neoplasm at T10-L1. The patient underwent radical resection of the tumor. Pathology and immunohistochemical study demonstrated a CCM. Unfortunately, the patient had a recurrence 5 years after the operation.

CONCLUSION: Intraspinal CCMs are very uncommon tumors. They usually show aggressive behavior with local recurrence observed in slightly more than half of all patients. We recommend serial imaging studies every 3 to 6 months during the first several years, after which an annual imaging study should be performed for follow-up.