January 2010 Archives

17 month old female with a cystic insular, temporal lesion

The patient is a 17 month old female with a cystic insular and temporal lobe lesion.  There is some enhancement.
DIG10x1.jpg dig2.jpg dig20x.jpg

dig40x.jpg



Desmoplastic infantile ganglioglioma (DIG)
- WHO grade I, glial or glioneuronal superficial tumors with prominent desmoplasia.
- Present in the first 2 years of life
- Supratentorium: Frontoparietal
- Symtoms of increased ICP
 
 Histopathology
- Distinctive features include collagenized regions dominated by spindly astrocytes in fascicular or storiform array and admixed small cells of primitive appearance in nodular aggregates.
 
Immunohistochemistry
- GFAP-positive astroglial cells within the desmoplastic regions
- Synaptophysin and Neurofilament positive neuronal cells in non-collagenized areas
- Small cell populations can be positive for GFAP, Neuronal markers ( Synaptophysin, Beta-tubulin, NF, MAP-2
 
 DDX 
  • Fibrohistiocytic neoplasms
  • Intracranial fibromatosis
  • Fibroblastic meningioma
  • Fibrosarcoma
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