Fact-sheet: Dysplastic cerebellar gangliocytoma
Updated on 04/24/2020 at 9:43 AM
View all RADEOS cases associated with this fact-sheet
Definition
Rare pseudotumor of the cerebellum, also known as Lhermitte-Duclos disease (first described by Lhermitte and Duclos in 1920).
The non-proliferative nature and absence of malignant transformation favor a hamartomatous origin, although the pathogenesis remains unknown.
Clinical features
Typically affects young adults.
May be asymptomatic when small.
Symptoms vary according to size:
- headaches
- nausea
- cerebellar syndrome
- cranial nerve palsy
- hydrocephalus
Possible association with:
- Cowden disease
- cerebral cortical malformations
- polydactyly
- hydromyelia
- macroglossia
Laboratory findings
Germline mutations in the PTEN tumor suppressor gene have been identified in some adult patients.
CT
Nonspecific hypodense cerebellar mass.
Calcifications possible.
MRI
Distortion of the laminar architecture of the cerebellum, giriform-appearing mass.
T1: iso/hypointense
T2: hyperintense with iso/hypointense striations
Diffusion: possible hyperintensity due to T2 effect but no restriction on ADC
T1 with contrast injection: generally no enhancement, possible mild enhancement related mainly to venous vascular structures
Management
Decompressive surgical management