J Neurosurg 78:287-289, 1993 Childhood meningioma associated with meningioangiomatosis Case report DEBORAH BLUMENTHAL, M.D., MARIANA BERHO, M.D., STEPHEN BLOOMFIELD, M.D., SYDNEY S. SCHOCHET, JR., M.D., AND HOWARD H. KAUFMAN, M.D. Departments of Neurosurgery and Pathology, West Virginia University Health Sciences Center, Morgantown, West Virginia v, This paper reports a childhood meningioma in association with meningioangiomatosis. The patient was an 1l-month-old baby boy who presented with a left focal seizure. He had no stigmata of neurofibromatosis. Computerized tomography and magnetic resonance imaging revealed an extra-axial, contrast-enhancing mass in the interhemispheric fissure which indented the right frontal lobe. The tumor was totally removed. Microscopically, the lesion was a fibrous and transitional meningioma with foci of necrosis and scattered mitotic figures. The adherent neural parenchyma showed the histological features of meningioangiomatosis. It is concluded that meningioangiomatosis may accompany childhood meningiomas more often than is generally appreciated. KEY WORDS 9 meningioangiomatosis ' meningioma neurofihromatosis 9 children M ENINGIOANGIOMATOSIS is generally regarded as a hamartomatous meningovascular lesion usually associated with neurofibromatosis. However, a few cases have been reported to have oc- curred in individuals without recognized neurofibro- matosis) ,7,8,1~ Meningiomas are infrequent in child- hood, comprising only 0.4% to 3.5% of all intracranial tumors in pediatric patients),* We are aware of only a very few cases of meningiomas definitely occurring in association with meningioangiomatosis, and none in a patient as young as the one we present here. Case Report This 9-month-old white baby boy had a normal gestation and development, with no significant medical history. He was admitted after a seizure consisting of tonic-clonic movements of his left upper and lower extremities that lasted 5 to 10 minutes and resolved spontaneously. Examination. Neurological examination was nor- mal for his age and he had no stigmata of neurofibro- matosis. There was no family history of abnormal skin lesions, seizures, or mental retardation. Computerized tomography (CT) and magnetic resonance imaging re- vealed a focally calcified enhancing lesion in the inter- hemispheric fissure which indented the right frontal lobe (Fig. 1). Dexamethasone and carbamazepine were prescribed, and the patient has had no further seizures since taking the medication. Operation. A CT-guided needle biopsy with twist- FIG. 1. Left: Contrast-enhanced axial computerized to- mography scan showing the unevenly enhanced neoplasm indenting the right frontal lobe, with surrounding edema. Right: Contrast-enhanced T~-weighted coronal magnetic res- onance image demonstrating the unevenly enhanced neo- plasm within the interhemispheric fissure. J. Neurosurg. / Volume 78 /February, 1993 287