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Choroid Plexus Xanthogranuloma

Summary

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  • Rare, benign intracranial lesion arising from the choroid plexus
  • Characterised by accumulation of lipid-laden macrophages (xanthoma cells)
  • Typically asymptomatic and incidentally discovered on imaging or autopsy

Pathophysiology

  • Exact etiology remains unclear
  • Hypothesized mechanisms:
    • Chronic inflammation of choroid plexus epithelium
    • Accumulation of lipids due to breakdown of red blood cells
    • Possible association with metabolic disorders or hyperlipidaemia
  • Histologically characterised by:
    • Foamy macrophages (xanthoma cells)
    • Cholesterol clefts
    • Haemosiderin deposits
    • Multinucleated giant cells

Demographics

  • Rare condition, true incidence unknown
  • More commonly found in adults, particularly in the elderly
  • No significant gender predilection reported
  • Increased prevalence in autopsy studies (up to 1.6-7% of cases)

Diagnosis

  • Often asymptomatic and discovered incidentally
  • When symptomatic, may present with:
    • Headaches
    • Dizziness
    • Cognitive decline
    • Focal neurological deficits (rare)
  • Definitive diagnosis requires histopathological examination

Imaging

  • CT findings:
    • Hyperdense lesion within the choroid plexus
    • May show calcifications
  • MRI findings:
    • T1-weighted: Hyperintense due to lipid content
    • T2-weighted: Variable signal intensity
    • Gradient echo: May show susceptibility artefacts due to haemosiderin
    • Contrast enhancement: Typically minimal to none
  • Differential diagnosis:
    • Choroid plexus papilloma
    • Meningioma
    • Metastasis

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  • The choroid plexus within the trigones of the lateral ventricles were expanded and hyperintense on diffusion-weighted imaging.

Treatment

  • Asymptomatic lesions:
    • Conservative management with regular imaging follow-up
  • Symptomatic lesions:
    • Surgical resection may be considered
    • Complete resection generally curative
  • Post-treatment follow-up:
    • Regular imaging to monitor for recurrence
    • No standardized follow-up protocol due to rarity of the condition

Differential diagnosis

Differential Diagnosis Differentiating Feature
Choroid plexus papilloma Lacks calcification; typically enhances more homogeneously
Meningioma Usually extra-ventricular; dural tail sign may be present
Ependymoma More heterogeneous appearance; may have cystic components
Metastasis Multiple lesions; irregular enhancement; surrounding oedema; no fat or calcium signal
Choroid plexus carcinoma More aggressive appearance; invasion of surrounding tissue
Intraventricular haemorrhage Acute onset; fluid-fluid levels on imaging
Subependymoma Typically in lateral ventricle angles; less enhancement
Central neurocytoma Usually attached to septum pellucidum; calcifications common
Intraventricular tuberculoma Concomitant basal meningitis; ring enhancement