9412/1 Desmoplastic infantile ganglioglioma



Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72

A rare tumour that occurs almost exclusively in infants, characterized histologically by a prominent desmoplastic stroma and a neuroepithelial population consisting mainly of neoplastic astrocytes (desmoplastic infantile astrocytoma, DIA) often together with a variable neuronal component (desmoplastic infantile ganglioglioma, DIG), in addition to aggregates of poorly differentiated cells, which are present in both.
The tumour typically involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although it presents clinically as large cystic lesion, it is biologically benign (WHO grade I) and generally has a good prognosis following surgical resection 1
 
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds.)
WHO Classification of Tumours of the Central Nervous System.
4th Edition
International Agency for Research on Cancer: Lyon 2007



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