SUMMARY:We report an unusual case of atypical cystic meningioma of the trigeminal nerve proved by histology in a 15-year-old white girl. A review of the literature showed that this is only the second reported case of a meningioma of the trigeminal nerve without dural attachment and the first occurrence in a pediatric patient. P rimary tumors of the trigeminal nerve and Meckel cave represent less than 0.5% of intracranial tumors.1 We report the case of a dumbbell-shaped atypical meningioma of the trigeminal nerve without dural attachment, involving the Meckel cave and the posterior fossa in an adolescent.
Case ReportA 15-year-old white girl presented with a 2-month history of leftsided facial numbness and loss of sensation of the left hemitongue. Physical examination showed hypoesthesia in the left V1, V2, and V3 dermatome distribution and absent left corneal reflex. She had no family history of neurofibromatosis, and physical examination revealed no evidence of café au lait spots or freckling in the axillary or inguinal area. Aside from the absence of a left corneal reflex, the findings of the ophthalmologic examination were unremarkable, with no evidence of lens abnormalities. A brain MR imaging revealed an extra-axial dumbbell-shaped cystic mass, without obvious "dural tail" or dural attachment, demonstrating peripheral rim enhancement (Fig 1), with a cystic component in the posterior fossa at the root entry zone of the left trigeminal nerve, and a second cystic lesion with a fluid-fluid level at the level of the left Meckel cave. The mass was thought to be consistent with a cystic schwannoma of the left trigeminal nerve. There were no masses along the course of the 8 cranial nerves.A 1-stage surgical excision of the middle and posterior fossa components of the mass was planned. The patient underwent a left-sided skull base craniotomy, with a left temporal craniotomy and zygomatic osteotomy, by using an extradural approach. At surgery, it was noted that the mass arose from the left trigeminal nerve, did not have a dural attachment, and was heavily intermixed with the surrounding trigeminal nerve. Surgical resection of the mass was thought to be complete.The resected gross specimen consisted of multiple fragments of brown-red variegated tissue. Histology (Fig 2) showed concentric structures typical of meningioma, hypercellularity, mitotically active foci, and focal necrosis, making it atypical. Embedded nerve fascicles of the excised trigeminal nerve appeared infiltrated by tumor. Reticulin stain showed chiefly perivascular staining in the tumor, highlighting its vascularity. A final diagnosis of atypical meningioma was made.Given the histology and the location of the neoplasm, we planned to treat residual or recurrent tumor with stereotactic radiosurgery. Since surgery, a postoperative MR imaging and subsequent follow-up MR imaging performed every 2 months in the last 4 months did not demonstrate evidence of macroscopic residual or recurrent tumor.Findings of an MR imaging of the cervical spine performed 2 mon...