DESMOPLASTIC INFANTILE GANGLIOGLIOMA

WHO Grade I

Cell of Origin: Large, mature neurons + glial component

Synonyms: Together with superficial cerebral astrocytoma may be referred to as desmoplastic supratentorial neuroepithelial tumors of infancy

Common Locations: Large, hemispheric superficial tumors

Demographics: Early infancy

Clinical Presentation: Macrocephaly

Histology: Glial and neuronal differentiation. Moderate pleomorphism; infrequent mitoses. Abundant desmoplasia characteristic.

Special Stains: Neuronal component confirmed by synaptophysin or electron microscopy. GFAP positive.

Progression: Good prognosis with surgical excision.

Radiology: Very large mass with cystic and solid components. The solid portion, which tends to be superficial, enhances intensely.

Comments: Related to superficial cerebral astrocytoma, which is distinguished only by its lack of neuronal elements.