Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma) - Review of the literature

WOS: 000246855900012Objectives: Lhermitte-Duclos disease (LDD), or dysplastic gangliocytoma of the cerebellum is a rare benign unilateral mass of the cerebellar cortex, characterized by a disarrangement of the normal cerebellar laminar cytoarchitecture and circumscribed enlargement of cerebellar folia. LDD was recently considered to be part of a multiple hamartoma-neoplasia syndrome [Cowden disease (CD)]. The debate whether LDD represents a neoplastic or hamartomatous lesion is still in progress. Methods: The aim of the present study is to answer this question with review of the literature emphasize on clinical presentation, radiologic findings, surgical procedures, and histopathologic features of LDD. Results: LDD most frequently presents in the third and fourth decades of life, but the age at clinical manifestation ranges from the neonatal period to the seventh decade. The initial presentation of LDD, similar to other posterior fossa tumors, includes increased intracranial pressure, vomiting, intermittent headache, cerebellar dysfunction, and noncommunicating hydrocephalus. Magnetic resonance imaging is the diagnostic modality and reveals characteristic usually nonenhancing gyriform patterns with enlargement of cerebellar folia. Surgical excision is a therapeutic procedure generally performed. The histopathologic findings of LDD include thickening of the molecular layer, which is occupied by abnormal ganglion cells, absence of the Purkinje cell layer, and hypertrophy of the granule cell layer. Conclusions: LDD is an unusual hamartomatous lesion of the cerebellar cortex, which can be associated with CD. When the diagnosis of either one of these 2 disorders is established, it is imperative to search for the other disease, to detect early malignant lesions that occur in CD