Tuberous sclerosis - subependymal giant cell astrocytoma

Case contributed by Hugo Andrés Bustamante
Diagnosis almost certain

Presentation

Background of tuberous sclerosis and intracardiac rhabdomyosarcoma, 2 day history of generalised tonic-clonic seizures.

Patient Data

Age: 10 years
Gender: Female
This study is a stack
Axial
non-contrast
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Axial bone
window
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Axial C+
arterial phase
This study is a stack
Axial C
+ Venous
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Info

At the level of the third ventricle, extending to the lateral ventricles, a heterogeneous density lesion with well defined, lobulated borders is observed demonstrating heterogeneous enhancement, with resultant dilation of the supratentorial ventricular system.

Diffuse subcortical multiple hypodense, poorly defined foci are also identified and do not demonstrate any enhancement.

Multiple periventricular subependymal nodules, some with pinpoint calcifications.

Bilateral Galassi I arachnoid cysts and left frontal arachnoid cyst.

Mega cisterna magna.

This study is a stack
Axial
T1
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Axial
T2
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Axial
FLAIR
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Axial
Gradient Echo
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Axial
T1 C+
This study is a stack
Axial
BRAVO
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Axial
DWI
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Axial
ADC
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Coronal
T2
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Coronal
T1 C+
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Sagittal
T1 FLAIR
This study is a stack
Sagittal
T1 C+
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Info

At the level of the third ventricle extending to the lateral ventricles, a lesion with defined lobulated edges is observed, isointense in T1 and T2, hyperintense in FLAIR with heterogeneous post-contrast enhancement, without diffusion restriction. There is associated supratentorial hydrocephalus.

Periventricular subependymal hamartomas with blooming artifact on gradient echo.

Multiple poorly defined areas are identified at the subcortical level, hyperintense on T2 and FLAIR, of diffuse distribution that does not enhance on post contrast sequences.

Bilateral Galassi I arachnoid cysts and left frontal arachnoid cyst.

Mega cisterna magna.

Case Discussion

A 10-year-old patient with a history of tuberous sclerosis and intracardiac rhabdomyosarcoma, consultation for a 2-day clinical picture consisting of generalised tonic-clonic seizures.

Taking into account the clinical history and the CT and MRI findings, it is concluded that it is a typical case of tuberous sclerosis and subependymal giant cell astrocytoma.

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