The patient is a 17 month old female with a cystic insular and temporal lobe lesion. There is some enhancement.
Fibrohistiocytic neoplasms
Intracranial fibromatosis
Fibroblastic meningioma
Fibrosarcoma
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
