Papillary endothelial hyperplasia associated with cortical dysplasia.
We report a unique case of papillary endothelial hyperplasia (PEH) presenting as a subcortical mass lesion intimately associated with focal cortical dysplasia (CD) and consider a possible causal relationship. A 6-year old girl presented with a 6-month history of a painless, frontoparietal skull "bump" associated with slowly progressive localised bossing followed by a 4-month history of absence attacks. Magnetic resonance imaging (MRI) revealed an adjacent parietal enhancing mass lesion beneath abnormal appearing cortex. A haemorrhagic vascular lesion with histology consistent with that of papillary endothelial hyperplasia was completely resected. Biopsies of the adjacent cortex showed CD. The patient has been symptom free post-surgery for 12 months with no MRI evidence of recurrence. Intracranial PEH is very rare and, in contrast to extracranial examples, half of the reported cases lacked a demonstrable vascular origin. Given that CD may be associated with intrinsic capillary hypervascularity, vascular malformations and tumours (e.g. dysembryoplastic neuroepithelial tumour) of a potential hypervascular or haemorrhagic nature, the association between PEH and CD may not be incidental. The abnormal vascularity not uncommonly found in CD may predispose to haemorrhage and/or thrombosis, the organisation of which may rarely be complicated by PEH. Alternatively, PEH and CD may both represent local, independent complications of a pre-existing vascular event or trauma.
du Plessis
Daniel G
DG
Department of Neuropathology, Walton Centre for Neurology and Neurosurgery, Lower Lane, Liverpool L9 7LJ, UK. duplessi-d@wcnn-tr.nwest.nhs.uk
Balamurali
Gopalakrishnan
G
Smith
E Trevor S
ET
Broome
John C
JC
Pizer
Barry L
BL
Mallucci
Conor L
CL
D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology*; D004730:Endothelium, Vascular / Q000473:pathology*; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008279:Magnetic Resonance Imaging; D011859:Radiography