Tuberous sclerosis with subependymal giant cell astrocytoma

Case contributed by Amro Omar
Diagnosis almost certain

Presentation

Seizures, mental retardation.

Patient Data

Age: 12 years

MRI demonstrates a large lobulated intensely enhancing mass arising from the left foramen of Monro causing mild hydrocephalus. Please note the subependymal nodules (best appreciated in the susceptibility weighted sequences), and the subtle hyperintensities in the occipital lobe representing a nice example of cortical/subcortical tubers of tuberous sclerosis (best appreciated on FLAIR).

Same patient four years earlier.

Case Discussion

Subependymal giant cell astrocytoma is:

  • WHO grade I
  • the most common CNS neoplasm in tuberous sclerosis (up to 15% of patients with TSC, and rarely (if ever) arises in absence of tuberous sclerosis)
  • typically occurs during the 1st two decades

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