DESMOPLASTIC INFANTILE GANGLIOGLIOMA (DIG)

From WikiCNS
(Difference between revisions)
Jump to: navigation, search
 
Line 1: Line 1:
Definition: DIG is a large, cystic tumor of infancy that involves the superficial cerebral cortex and leptomeninges, and is composed of neoplastic astrocytes and neuronal components within a prominent desmoplastic stroma. DIG is considered a WHO Grade I tumor.
+
{{appr}}
 +
{{TOCRight}}
  
Epidemiology: DIG is a rare tumor of infancy. The age range extends from 1-24 months, though non-infantile cases have been reported. There is a male predominance (1.7:1). DIG may be associated with cortical dysplasia.
+
===Definition===
 +
DIG is a large, cystic tumor of infancy that involves the superficial cerebral cortex and leptomeninges, and is composed of neoplastic astrocytes and neuronal components within a prominent desmoplastic stroma. DIG is considered a WHO Grade I tumor.
  
Localization: DIG is a supratentorial lesion that commonly involves more than one lobe.
+
===Epidemiology===
 +
DIG is a rare tumor of infancy. The age range extends from 1-24 months, though non-infantile cases have been reported. There is a male predominance (1.7:1). DIG may be associated with cortical dysplasia.
  
Clinical presentation: Symptoms are of short duration and include findings consistent with increased intracranial pressure.
+
===Localization===
 +
DIG is a supratentorial lesion that commonly involves more than one lobe.
  
Imaging: MRI demonstrates a hypointense cytic mass with an isointense peripheral solid component that shows contrast-enhancement.
+
===Clinical presentation===
 +
Symptoms are of short duration and include findings consistent with increased intracranial pressure.
  
Macroscopy: DIG 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.
+
===Imaging===
 +
MRI demonstrates a hypointense cytic mass with an isointense peripheral solid component that shows contrast-enhancement.
  
Histopathology: DIG is composed of a variable mix of neoplastic astrocytes and neuronal elements 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.
+
===Macroscopy===
 +
DIG 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.
  
Molecular genetics: The molecular genetics of DIG have not been elucidated.
+
===Histopathology===
 +
DIG is composed of a variable mix of neoplastic astrocytes and neuronal elements 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.
  
Prognosis: DIG is a surgically curable disease.
+
===Molecular genetics===
 +
The molecular genetics of DIG have not been elucidated.
 +
 
 +
===Prognosis===
 +
DIG is a surgically curable disease.
  
 
[[Category: Neurosurgery]]
 
[[Category: Neurosurgery]]

Latest revision as of 18:16, 10 July 2008

Checkmark.gif This article has been reviewed by the NeuroWiki Editorial Board


Contents

[edit] Definition

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

[edit] Epidemiology

DIG is a rare tumor of infancy. The age range extends from 1-24 months, though non-infantile cases have been reported. There is a male predominance (1.7:1). DIG may be associated with cortical dysplasia.

[edit] Localization

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

[edit] Clinical presentation

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

[edit] Imaging

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

[edit] Macroscopy

DIG 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.

[edit] Histopathology

DIG is composed of a variable mix of neoplastic astrocytes and neuronal elements 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.

[edit] Molecular genetics

The molecular genetics of DIG have not been elucidated.

[edit] Prognosis

DIG is a surgically curable disease.

Personal tools