Case 3 -

Meningioangiomatosis Elisabeth Rushing Armed Forces Institute of Pathology Washington, DC

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Click here to download handout in 1-up pdf format for the current section (2.39 MB) Click here to download handout in 6-up pdf format for the current section (1.02 MB) History The patient is a 9 year-old boy who has had seizures since age 2, at which time he was discovered to have a right frontal mass. The seizures were controlled with anticonvulsant medication until age 8, when the frequency increased to several per week. The patient's mother also reported behavioral disturbances that paralleled the increased number of seizures. Coronal T2 weighted image demonstrates low signal along the cortex of the right frontal lobe and the morphology of the cortex is abnormal. The subjacent white matter reveals T2 hyperintensity. On the axial post-contrast T1 weighted image, contrast enhancement is noted in the region of the abnormal cortical signal on T2. Intraoperative findings are that of a firm, discrete lesion that follows the contours of the cortical ribbon. The cut sections of the lesion are gritty. Case 3 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 3 - Figure 1 Clinical history Case 3 - Figure 2 (a) Coronal T2 weighted image demonstrates low signal along the cortex of the right frontal lobe and the morphology of the cortex is abnormal. The subjacent white matter reveals T2 hyperintensity. (b) On the axial post-contrast T1 weighted image, contrast enhancement is noted in the region of the abnormal cortical signal on T2. Case 3 - Figure 3 Intraoperative findings are that of a firm, discrete mass that follows the contours of the cortical ribbon. Case 3 - Figure 4 Spindle cell and angiomatous microvascular proliferation permeate the cortex. Case 3 - Figure 5 Spindle cells ensheathe blood vessels; psammoma bodies are present. Case 3 - Figure 6 In some areas, only small islands of uninvolved cortex remain. Case 3 - Figure 7 Neurons and gliosis are seen in the intervening cortex Case 3 - Figure 8 Disorganized cortex with dysplastic-appearing neurons. Case 3 - Figure 9 Thickened leptomeninges containing numerous blood vessels are flanked by gyri with exuberant spindle-cell proliferation and psammoma bodies. Case 3 - Figure 10 Discrete interface between involved cortex and white matter. Differential Diagnosis Vascular malformation Meningioma Schwannoma Ganglion cell tumor Final Diagnosis Meningioangiomatosis Key words: meningioma,neurofibromatosis II, vascular malformation Take-home bullet points Low-power examination is the key to recognizing the characteristic histopathologic features Immunohistochemical studies contribute little to the practical diagnosis Sporadic and inherited forms of this entity differ clinically and histologically References Giangaspero F, Guiducci AN, Lenz FA, Mastronardi L, Burger PC (1999) Meningioma with meningioangiomatosis: A condition mimicking invasive meningiomas in children and young adults. Report of two cases and review of the literature. Am J Surg Pathol 23:872-875. Goates JJ, Dickson DW, Horoupian DS (1991) Meningioangiomatosis: an immunocytochemical study. Acta Neuropathol 82:527-532. Halper J, Scheithauer BW, Okazaki H, Laws, ER Jr. (1986) Meningioangiomatosis: A report of six cases with special reference to the occurence of neurofibrillary tangles. J Neuropathol Exp Neurol 45:426-446. Lopez J, Ereno C, Oleaga L, Areitio E (1996) Meningioangiomatosis and oligodendroglioma in a 15-year-old boy. Arch Pathol Lab Med 120:587-590. Meltzer CC, Liu AY, Perrone AM, Hamilton RL (1998) Meningioangiomatosis: MR imaging with histopathologic correlation. Am J Radiol 170:804-805. Paulus W, Peiffer J, Roggendorf W (1989) Meningio-Angiomatosis. Path Res Pract 184:446-452. Prayson RA (1995) Meningioangiomatosis: A clinicopathologic study including MIB1 immunoreactivity. Arch Pathol Lab Med 119:1061-1064. Perry A, Kurtkaya-Yapicier O, Scheithauer BW, Robinson S, Prayson RA, Kleinschmidt-DeMasters BK, Stemmer-Rachamimov AO, Gutmann DH (2005) Insights into meningioangiomatosis with and without meningioma: a clinicopathologic and genetic series of 24 cases with review of the literature. Brain Pathol;15:55-65. Sinkre P, Perry A, Cai D, Raghavan R, Watson M, Wilson K, Barton Rogers B (2001) Deletion of the NF2 region in both meningioma and juxtaposed meningioangiomatosis: Case report supporting a neoplastic relationship. Pediatr Dev Pathol 4:568-572. Stemmer-Rachamimov AO, Horgan MA, Taratuto AL, Munoz DG, Smith TW, Frosch MP, Louis DN (1997) Meningioangiomatosis is associated with neurofibromatosis 2 but not with somatic alterations of the NF2 gene. J Neuropathol Exp Neurol 56:485-489.