9493/0 Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos)


Definitions

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

A rare, benign cerebellar mass that occurs in young adults and is composed of dysplastic ganglion cells (WHO grade I 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



). It is a major CNS manifestation of Cowden disease , an autosomal dominant condition that causes a variety of hamartomas and neoplasms.