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170 - Subependymal Giant Cell Astrocytoma (SEGA)

from Section 6 - Primarily Intra-Axial Masses

Published online by Cambridge University Press:  05 August 2013

Andrea Rossi
Affiliation:
Children’s Research Hospital, Genoa, Italy
Zoran Rumboldt
Affiliation:
Medical University of South Carolina
Mauricio Castillo
Affiliation:
University of North Carolina, Chapel Hill
Benjamin Huang
Affiliation:
University of North Carolina, Chapel Hill
Andrea Rossi
Affiliation:
G. Gaslini Children's Research Hospital
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Summary

Specific Imaging Findings

Subependymal giant cell astrocytomas (SEGAs) almost exclusively originate from the subependymal surface of the caudate nucleus, near the foramen of Monro, in patients with tuberous sclerosis complex (TSC). Bilateral lesions are common; they are often asymmetric both in size and growth rate. SEGAs are prevailingly solid, although cystic components may be present, especially in large masses. Lesions may show lobulated margins and resemble a conglomeration of nodules. Calcification is common, and is best seen on CT. The solid component is isointense with gray matter on all MR imaging sequences, and enhances homogeneously with gadolinium administration. The lesions are similar to the brain on diffusion imaging. Diameter over 12 mm, or a lesion more than 5 mm in size with growth on serial imaging are the characteristics indicative of SEGA. All lesions at the foramen of Monro in TSC patients should be presumed to be SEGA until proven otherwise. Contrast enhancement was considered a differentiating feature of SEGA on CT; however, many subependymal nodules enhance on MRI.

Pertinent Clinical Information

SEGA is found predominantly during the first and second decades of life, sometimes even in utero. The lesion may be asymptomatic per se; however, when sufficiently large it may obstruct CSF flow at the foramen of Monro, leading to elevated intracranial pressure and hydrocephalus. Worsening of epilepsy may lead to neuroimaging and diagnosis of SEGA. Neurosurgical resection is an effective standard treatment and may be performed before the lesions become symptomatic; however, SEGAs frequently recur.

Type
Chapter
Information
Brain Imaging with MRI and CT
An Image Pattern Approach
, pp. 351 - 352
Publisher: Cambridge University Press
Print publication year: 2012

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References

1. Baron, Y, Barkovich, AJ. MR Imaging of tuberous sclerosis in neonates and young infants. AJNR 1999;20:907–16.Google ScholarPubMed
2. Raju, GP, Urion, DK, Sahin, M. Neonatal subependymal giant cell astrocytoma: new case and review of literature. Pediatr Neurol 2007;36:128–31.CrossRefGoogle ScholarPubMed
3. Grajkowska, W, Kotulska, K, Jurkiewicz, E, et al.Subependymal giant cell astrocytomas with atypical histological features mimicking malignant gliomas. Folia Neuropathol 2011;49:39–46.Google ScholarPubMed
4. Campen, CJ, Porter, BE. Subependymal Giant Cell Astrocytoma (SEGA) treatment update. Curr Treat Options Neurol 2011;13:380–5.CrossRefGoogle ScholarPubMed
5. Krueger, DA, Care, MM, Holland, K, et al.Everolimus for subependymal giant-cell astrocytomas in tuberous sclerosis. N Engl J Med 2010;363:1801–11.CrossRefGoogle ScholarPubMed

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