DESMOPLASTIC INFANTILE ASTROCYTOMA (DIA)

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Definition: DIA is a large, cystic tumor of infancy that involves the superficial cerebral cortex and leptomeninges, and is composed of neoplastic astrocytes within a prominent desmoplastic stroma. DIA is considered a WHO Grade I tumor.
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===Definition===
 +
DIA is a large, cystic tumor of infancy that involves the superficial cerebral cortex and leptomeninges, and is composed of neoplastic astrocytes within a prominent desmoplastic stroma. DIA is considered a WHO Grade I tumor.
  
Epidemiology: DIA represents nearly 16% of all intracranial tumors in infants. The age range extends from 1-24 months, though non-infantile cases have been reported. There is a male predominance (1.7:1). DIA may be associated with cortical dysplasia.
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===Epidemiology===
 +
DIA represents nearly 16% of all intracranial tumors in infants. The age range extends from 1-24 months, though non-infantile cases have been reported. There is a male predominance (1.7:1). DIA may be associated with cortical dysplasia.
  
Localization: DIA is a supratentorial lesion that commonly involves more than one lobe.
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===Localization===
 +
DIA is a supratentorial lesion that commonly involves more than one lobe.
  
Clinical presentation: Symptoms are of short duration and include findings consistent with increased intracranial pressure.
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===Clinical presentation===
 +
Symptoms are of short duration and include findings consistent with increased intracranial pressure.
  
Imaging: MRI demonstrates a hypointense cytic mass with an isointense peripheral solid component that shows contrast-enhancement.
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===Imaging===
 +
MRI demonstrates a hypointense cytic mass with an isointense peripheral solid component that shows contrast-enhancement.
  
Macroscopy: DIA are large tumors composed of deep multi-loculated cysts filled with clear or xanthochromic fluid. The superficial component is primarily extracerebral and is commonly attached to the dura.
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===Macroscopy===
 +
DIA are large tumors composed of deep multi-loculated cysts filled with clear or xanthochromic fluid. The superficial component is primarily extracerebral and is commonly attached to the dura.
  
Histopathology: DIA is composed of neoplastic astrocytes within a prominent, reticulin-rich desmoplastic stroma. There is a sharp demarcation between the tumor and the cortical surface, though Virhcow-Robin spaces in the underlying cortex are often filled with tumor cells.
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===Histopathology===
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DIA is composed of neoplastic astrocytes within a prominent, reticulin-rich desmoplastic stroma. There is a sharp demarcation between the tumor and the cortical surface, though Virhcow-Robin spaces in the underlying cortex are often filled with tumor cells.
  
Molecular genetics: The molecular genetics of DIA have not been elucidated.
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===Molecular genetics===
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The molecular genetics of DIA have not been elucidated.
  
Prognosis: DIA is a surgically curable disease.
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===Prognosis===
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DIA is a surgically curable disease.
  
 
[[Category: Neurosurgery]]
 
[[Category: Neurosurgery]]

Revision as of 18:14, 10 July 2008

Contents

Definition

DIA is a large, cystic tumor of infancy that involves the superficial cerebral cortex and leptomeninges, and is composed of neoplastic astrocytes within a prominent desmoplastic stroma. DIA is considered a WHO Grade I tumor.

Epidemiology

DIA represents nearly 16% of all intracranial tumors in infants. The age range extends from 1-24 months, though non-infantile cases have been reported. There is a male predominance (1.7:1). DIA may be associated with cortical dysplasia.

Localization

DIA is a supratentorial lesion that commonly involves more than one lobe.

Clinical presentation

Symptoms are of short duration and include findings consistent with increased intracranial pressure.

Imaging

MRI demonstrates a hypointense cytic mass with an isointense peripheral solid component that shows contrast-enhancement.

Macroscopy

DIA are large tumors composed of deep multi-loculated cysts filled with clear or xanthochromic fluid. The superficial component is primarily extracerebral and is commonly attached to the dura.

Histopathology

DIA is composed of neoplastic astrocytes within a prominent, reticulin-rich desmoplastic stroma. There is a sharp demarcation between the tumor and the cortical surface, though Virhcow-Robin spaces in the underlying cortex are often filled with tumor cells.

Molecular genetics

The molecular genetics of DIA have not been elucidated.

Prognosis

DIA is a surgically curable disease.

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