desmoplastic infantile ganglioglioma / desmoplastic infantile astrocytoma (DOID:0081259)
Alliance: disease page
Synonyms: Desmoplastic Infantile Astrocytoma; desmoplastic infantile ganglioglioma; desmoplastic infantile ganglioglioma and desmoplastic infantile astrocytoma
Alt IDs: NCI:C4738, NCI:C9476
Definition: A ganglioglioma occurring predominantly in the cerebral hemispheres of infants, that are driven by MAPK pathway activation and composed of a mixed astrocytic and neuronal component (DIG) or an astrocytic component only (DIA) embedded in an extensive desmoplastic stroma, often containing foci of undifferentiated embryonal-like tumour cells.