desmoplastic infantile ganglioglioma / desmoplastic infantile astrocytoma

Term ID

DOID:0081259

Synonyms

• Desmoplastic Infantile Astrocytoma
• desmoplastic infantile ganglioglioma
• desmoplastic infantile ganglioglioma and desmoplastic infantile astrocytoma

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. (2)

References

• ICDO:9412/1
• NCI:C4738
• NCI:C9476

Ontology

Human Disease   ( DOID:0081259 )