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Int J Clin Exp Pathol 2013;6(10):2267-2271
Encephalomalacic dysplastic mass lesion associated with vascular abnormalities in an
elderly man. Is this “acquired” focal cortical dysplasia?
Hidehiro Takei, Meenakshi B Bhattacharjee
Department of Pathology and Genomic Medicine, Houston Methodist Hospital, 6565 Fannin Street, Houston, TX 77070, USA; Department of
Pathology and Laboratory Medicine, The University of Texas Health Science Center at Houston, TX, USA
Received July 31, 2013; Accepted August 17, 2013; Epub September 15, 2013; Published October 1, 2013
Abstract: Focal cortical dysplasia (FCD) is known to occur in association with a principal lesion and has been newly introduced as FCD type III
in the ILAE 2011 classification system of FCDs. FCD type IIIc is associated with vascular malformations, and in all such cases in the literature
with histologic confirmation, the principal lesion was a cavernous angioma. We present here a case of mass-forming FCD type IIIc with very
unusual vasculopathies. The patient, a 75-year-old man, presented with a seizure and left-sided weakness. MRI showed a right frontal intra-
axial mass (4 cm in diameter). He had significant multiple cardiovascular risk factors and a history of melanoma and bladder cancer.
Craniotomy was performed for excision of the suspected neoplasm. Histologically, the cortex showed transition from normal, laminar cortical
architecture to marked architectural abnormality with irregular areas of encephalomalacia, with intervening, almost nodular regions of
“preserved” cortex. These regions consisted of large clusters of dysplastic and maloriented neurons. There were medium-sized to larger
arteries with marked medial thickening and luminal stenosis, predominantly within the markedly thickened and fibrosed leptomeninges. In
addition, the parenchymal vessels showed markedly hyalinized and thickened walls, and several exhibited glomeruloid clusters of new
capillaries. We interpreted the cortical and subcortical encephalomalacic features to be presumably “secondary” to the underlying/associated
vascular pathology. This case may represent “acquired” FCD. It is possible that this patient had a preexistent cortical dysplasia with secondary
ischemic changes; however, given the absence of previous history of seizure, this appears less likely. (IJCEP1307056).
Keywords: Focal cortical dysplasia type IIIc, encephalomalacia, vascular malformation, acquired FCD
Address correspondence to: Dr. Hidehiro Takei, Department of Pathology and Genomic Medicine, Houston Methodist Hospital, 6565 Fannin
Street, Houston, TX 77070, USA. Tel: 713-441-6959; Fax: 713-793-1603; E-mail: HMTakei@tmhs.org