Brain Tumors Research Today is a free monthly online journal that collates and summarizes the latest research about Brain Tumors, including details on symptoms, benign and malignant tumors, gliomas, screening, treatment.

Lhermitte-Duclos disease associated with Cowden syndrome.

Tan TC, Ho LC

Department of Neurosurgery, Queen Elizabeth Hospital, Kowloon, Hong Kong. tzechingtan@yahoo.com

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.

Published 19 June 2007 in J Clin Neurosci, 14(8): 801-5.
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