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Papillary endothelial hyperplasia associated with cortical dysplasia.

Daniel G du Plessis1, Gopalakrishnan Balamurali, E Trevor S Smith

  • 1Department of Neuropathology, Walton Centre for Neurology and Neurosurgery, Lower Lane, Liverpool L9 7LJ, UK. duplessi-d@wcnn-tr.nwest.nhs.uk

Acta Neuropathologica
|January 31, 2003
PubMed
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A rare brain tumor, papillary endothelial hyperplasia (PEH), was found with focal cortical dysplasia (CD) in a child. This case suggests a potential link between these conditions, possibly due to abnormal vascularity in CD.

Area of Science:

  • Neurology
  • Pathology
  • Pediatric Neurosurgery

Background:

  • Intracranial papillary endothelial hyperplasia (PEH) is an exceptionally rare vascular tumor.
  • Focal cortical dysplasia (CD) is a developmental malformation of the cerebral cortex often associated with epilepsy.

Observation:

  • A 6-year-old girl presented with a skull mass and absence seizures.
  • MRI revealed a subcortical mass lesion adjacent to abnormal-appearing cortex.
  • Surgical resection of the mass confirmed PEH; adjacent cortical biopsies showed CD.

Findings:

  • The resected lesion was a hemorrhagic vascular tumor consistent with PEH.
  • Histological examination of the adjacent cortex revealed focal cortical dysplasia.
  • The patient remained symptom-free post-surgery with no recurrence at 12 months.

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Implications:

  • The co-occurrence of PEH and CD suggests a potential causal relationship, challenging the notion that PEH always arises from a vascular origin.
  • Abnormal vascularity in CD may predispose to vascular events like hemorrhage or thrombosis, potentially leading to PEH formation.
  • This case highlights the importance of considering rare tumor associations in pediatric neurological disorders and may inform future diagnostic and therapeutic strategies.