Lhermitte-Duclos disease associated with Cowden syndrome.
Lhermitte-Duclos disease or dysplastic gangliocytoma of the cerebellum, is a rare cerebellar lesion, which can cause mass effects in the posterior fossa. It may occur sporadically, or in association with Cowden syndrome. Cowden syndrome or multiple hamartoma-neoplasia syndrome, is an uncommon autosomal dominant condition characterized by mucocutaneous lesions and systemic malignancies. We report two patients with Lhermitte-Duclos disease and associated Cowden syndrome. The clinical, radiological and histopathological features and management strategies of this rare disease complex are discussed.
Tan
Tze-Ching
TC
Department of Neurosurgery, Queen Elizabeth Hospital, Kowloon, Hong Kong. tzechingtan@yahoo.com
Ho
Luen-Cheung
LC
D002528:Cerebellar Neoplasms / Q000150:complications* / Q000473:pathology; D005260:Female; D005729:Ganglioneuroma / Q000150:complications* / Q000473:pathology; D006223:Hamartoma Syndrome, Multiple / Q000150:complications* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008875:Middle Aged