An aggressive extension of dumbbell-type pediatric skull base meningioma: a case report with a review of literature.

BACKGROUND: Pediatric meningiomas account for less than 2% of pediatric brain tumors. Pediatric clear cell meningiomas (CCM) occurring in the posterior fossa are particularly rare. Therapeutic strategies differ among the previous pediatric CCM case reports. Therefore, to clarify the clinical features of pediatric CCM, we report a rare case of dumbbell-type pediatric CCM and corresponding literature review. CASE DESCRIPTION: A 7-year-old boy with complaints of headache, left facial palsy, dysarthria, and left-sided ataxic gate was emergently admitted to our hospital. His consciousness level was slight stupor, GCS3-5-6, and he showed left ptosis, dysarthria, and ataxias of the left trunk and extremities. MRI showed acute obstructive hydrocephalus due to the tumor's compression of the brain stem. The dumbbell-shaped tumor extended from the lateral wall of the cavernous sinus, through the left Meckel's cave to the cerebellopontine angle. Physical examination and perioperative MRI showed no evidence regarding with neurofibromatosis types I or II. The tumor was removed in a 2-staged operation. Postoperative proton therapy was done to treat some residual tumors. One year after postoperative proton therapy, there is no recurrence, and, apart from left corneal and facial hypesthesia, he is healthy. CONCLUSIONS: We reported a rare case of pediatric skull base-type CCM with huge extension originating from the antero-medial wall of the Meckel's cave firmly adhered to the cavernous sinus wall to the posterior fossa that was successfully treated with surgery and postoperative proton therapy. CCM has a high recurrence rate, therefore, careful, prolonged follow up is needed.