Brain, Dysplastic gangliocytoma of cerebellum

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.