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Hypervascularity in Lhermitte-Duclos disease--case report

K Ishizaki1, G Daita, Y Yonemasu

  • 1Department of Neurosurgery, Asahikawa Medical College, Hokkaido.

Neurologia Medico-Chirurgica
|May 1, 1997
PubMed
Summary

Lhermitte-Duclos disease, a rare brain tumor, can cause gait problems. Surgical removal of this hypervascular tumor variant led to neurological improvement in a 61-year-old male patient.

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Area of Science:

  • Neuropathology
  • Neuroradiology
  • Neurosurgery

Background:

  • Lhermitte-Duclos disease, also known as dysplastic gangliocytoma, is a rare cerebellar tumor.
  • This case presents a hypervascular variant of Lhermitte-Duclos disease.
  • The patient, a 61-year-old male, exhibited gait disorder as the primary symptom.

Observation:

  • Imaging studies including computed tomography (CT) and magnetic resonance imaging (MRI) revealed contrast enhancement of the tumor.
  • Angiography confirmed the hypervascular nature of the tumor by demonstrating a distinct tumor stain.
  • Histopathological examination identified a unique double-layered structure with myelinated axons and dysplastic granular cells, alongside numerous dilated, thin-walled blood vessels.

Findings:

  • The hypervascular variant of dysplastic gangliocytoma was diagnosed.

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  • The tumor exhibited characteristic radiological features of contrast enhancement and a tumor stain.
  • Histology confirmed the specific layered architecture and vascularity of the lesion.

    • Implications:

    • Partial tumor resection was successfully performed.
    • The patient experienced resolution of neurological deficits, specifically the gait disorder, post-surgery.
    • This case highlights the importance of recognizing and surgically managing hypervascular Lhermitte-Duclos disease for favorable patient outcomes.