Brain, Dysplastic gangliocytoma of cerebellum [LMP18710]

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Image Details
Diagnostic Modality: 
Whole Slide Image
Disease Category: 
Neoplasia
Diagnosis: 
Dysplastic gangliocytoma of the cerebellum
Species: 
Human
Organ System: 
Central Nervous System
Age: 
30 years
Diagnosis Code: 
ICD-10: D33.1 | ICD-O: 9493/0
Slide Contributor Information
Gao, A., Munoz D.

Keywords

Lhermitte-Duclos disease, Cowden syndrome, PTEN

Clinical History

30-year-old man with a cerebellar mass on neuroimaging.

Clinical Discussion

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a rare entity associated with Cowden syndrome (mutations in PTEN tumour suppressor gene). Whether it is a hamartoma or a true neoplasm is still debated. Microscopically, there is diffuse enlargement and architectural distortion of the molecular and granular cell layers of the cerebellum which are filled by dysplastic ganglionic cells. A layer of myelinated fibres can sometimes be seen in the outer molecular layer. While the dysplastic gangliocytoma is usually sufficiently treated with surgical resection, patients should be screened for systemic malignancies and other features of Cowden syndrome.