An Aggressive Extension of Dumbbell-Type Pediatric Skull Base Meningioma: A Case Report with Review of the Literature

Abstract

Pediatric meningiomas account for less than 2% of pediatric brain tumors. Pediatric clear cell meningiomas (CCMs) 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 CCMs, we report a rare case of dumbbell-type pediatric CCM and a corresponding literature review. A 7-year-old boy with complaints of headache, left facial palsy, dysarthria, and left-sided ataxic gait was emergently admitted to our hospital. His consciousness level was slight stupor, with Glasgow Coma Scale score 3-5-6, and he showed left ptosis, dysarthria, and ataxias of the left trunk and extremities. Magnetic resonance imaging (MRI) scan showed acute obstructive hydrocephalus because of the tumor's compression of the brainstem. 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 scan showed no evidence regarding neurofibromatosis type 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. We reported a rare case of pediatric skull base-type CCM with huge extension originating from the anteromedial wall of 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.