AANP Diagnostic Slide Session Case 04 Tracie

AANP: Diagnostic Slide Session – Case 04 Tracie Pham, M. D. , William H. Yong, M. D. , Gary W. Mathern, M. D. , and Harry V. Vinters, M. D. UCLA Department of Pathology and Laboratory Medicine, Division of Neuropathology, and Department of Neurosurgery

Clinical History 13 year-old male who presents with complex partial seizures since age 6 years Initially managed on Trileptal, with Keppra added in 2005; currently on Lamictal History of NF 1, diagnosed at age 18 months due to presence of café-au lait skin lesions and neurofibromas

Radiologic Studies PET scan on 6/22/10: Small focus of hypometabolism in the right frontal lobe MRI scan on 9/30/10: Stable corticallybased lesion in the right anterior frontal convexity with small multifocal enhancement, small cystic foci and no perilesional edema. Stable small foci of T 2 W hyperintensity in the cerebrum.

MRI of Brain Scan 9/30/10 Image courtesy of Hassana Ibrahim, M. D.

MRI of Brain Scan 9/30/10 Image courtesy of Hassana Ibrahim, M. D.

MRI of Brain Scan 9/30/10 Image courtesy of Hassana Ibrahim, M. D.

Smooth Muscle Actin

Smooth Muscle Actin

Smooth Muscle Actin

GFAP

Neurofilament

Neu. N

Final Diagnosis Meningioangiomatosis Focus consistent with ganglioma (predominantly ganglion cells), WHO grade I

Diagnostic Points Both meningioangiomatosis (MA) and ganglioma can present with seizures Meningioangiomatosis associated with NF 2 (in this case, patient had NF 1) Tissue sampling is critical – focus of ganglioma only on one section Smooth muscle actin helpful in highlighting the microvascular component and smooth muscle cell proliferation of MA; interesting SMA immunoreactivity in cells with neuronal phenotype

References Prayson RA. Tumours arising in the setting of paediatric chronic epilepsy. Pathology 2010; 42(5): 426 -431 Stemmer-Rachamimov AO, Horgan MA, Taratuto AL, Munoz DG, Smith TW, Frosch MP et al. Meningioangiomatosis is associated with neurofibromatosis 2 but not with somatic alterations of the NF 2 gene [Review]. J Neuropathol Exp Neurol 1997; 56: 485 -489 Vinters HV. Pediatric Vascular Malformations. In: Pathology and Genetics. Developmental Neuropathology. Golden JA and Harding BN (eds). Basel: ISN Neuropath Press; 2004, pp. 116 -124 Wiebe S, Munoz DG, Smith S, and Lee DH. Meningioangiomatosis: A comprehensive analysis of clinical laboratory features. Brain 1999; 122: 709 -726