Rare. Benign tumor of the cerebellum (dysplastic gangliocytoma) the mass effect of which can cause headache, cerebellar ataxia or intracranial hypertension. This  disease especially affects women and occurs typically around the age of 30-40 years. Macrocephaly (> 95 percentile) is often present. It is frequently associated with Cowden syndrome (see this topic) which makes the patient at risk of multiple cancers (thyroid, endometrium, breast etc).

The MRI image is typical: "Tiger" image of the tumor in the cerebellum: these parallel streaks on the surface of the lesion probably represent dysplastic cerebellar folds.

Anesthetic implications:

management of a posterior fossa tumor with or without intracranial hypertension; risk of multiple cancers: possible sequelae of chemo- and/or radiotherapy.

References : 

-        Tan T-C, Ho L-C. 
Lhermitte-Duclos disease associated with Cowden syndrome. 
J Clin Neurosc 2007 ; 14 : 801-5.

Updated: February 2019