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Review
. 2018 Mar 28;29(3):295-301.
doi: 10.1515/revneuro-2017-0027.

The natural history of subependymal giant cell astrocytomas in tuberous sclerosis complex: a review

Denise L Chan  1 , Tessa Calder  2 , John A Lawson  1 , David Mowat  2 , Sean E Kennedy  1
Affiliations
Review

The natural history of subependymal giant cell astrocytomas in tuberous sclerosis complex: a review

Denise L Chan et al. Rev Neurosci. .

Abstract

Tuberous sclerosis complex (TSC) is an auto-somal-dominant inherited condition with an incidence of approximately 1:6000 births, characterised by deregulated mTOR activity with multi-site hamartomas. Subependymal giant cell astrocytomas (SEGA) are one such hamartoma, affecting up to 24% of patients with TSC. Their intraventricular location may lead to life-threatening obstructive hydrocephalus. Current management is hampered by a lack of understanding regarding the natural history, behaviour and growth patterns of SEGA. We review the current literature to summarise what is known about SEGA in the following areas: (1) diagnostic criteria, (2) prevalence, (3) origin, (4) imaging characteristics, (5) growth rate, (6) genotype-phenotype correlation, (7) congenital SEGA and (8) SEGA as a marker of severity of other TSC manifestations.

Keywords: MRI; SEGA; TSC; genotype; growth; neuroimaging.

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