Pineal region giant cell astrocytoma associated with tuberous sclerosis: case report.

Abstract:

:Tuberous sclerosis complex is a genetic disorder characterized by the development of hamartomas in multiple organs including the brain, skin, eye, kidney, and heart. Neurological features include seizures and mental retardation. Cortical tubers and subependymal nodules are the characteristic intracranial lesions of tuberous sclerosis. Subependymal giant cell astrocytomas, typically located adjacent to the foramen of Monro, can enlarge and cause symptomatic ventricular obstruction. The authors describe the case of a 3-year-old boy with a history of tuberous sclerosis and retinal lesions who presented with an enlarging enhancing pineal region mass. Via an infratentorial supracerebellar approach, the mass was removed using both the operative microscope and a rigid neuroendoscope. Pathological examination showed a giant cell astrocytoma. To the authors' knowledge, this is the first reported case of tuberous sclerosis associated with a giant cell astrocytoma of the pineal region. Diagnostic considerations are discussed.

journal_name

J Neurosurg

journal_title

Journal of neurosurgery

authors

Dashti SR,Robinson S,Rodgers M,Cohen AR

doi

10.3171/ped.2005.102.3.0322

subject

Has Abstract

pub_date

2005-04-01 00:00:00

pages

322-5

issue

3 Suppl

eissn

0022-3085

issn

1933-0693

journal_volume

102

pub_type

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