Magnetic resonance imaging in the diagnosis and treatment of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum)

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) and its relation to the multiple hamartoma syndrome (Cowden disease)

“…CT will identify a mass at the posterior fossa but it is unspecific in diagnosing this entity. MRI is the appropiate diagnostic tool since it is capable to show the characteristic foliated pattern of the lesion at the posterior fossa, an almost pathognomonic finding [12,15,17,24,27,28]. There is usually a mild surrounding edema of the huge intraaxial vermian or vermian-hemispheric lesion, which presents a minimal gadolinium enhancement and an absence of cystic or necrotic areas.…”

Uncommon glial tumors

“…Little data exist regarding radiation therapy or chemotherapy. MR may also be useful in determining the presence of residual tumor (Marano et al 1988;Reeder et al 1988;Smith et al 1989;Ashley et al 1990;Faillot et al 1990). corresponding to the pathologic finding of thickened cerebellar folia.…”

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)

“…[18][19][20][21][22][23] Prior to recent advances in neuroimaging and microsurgery, Lhermitte-Duclos tumours were associated with very poor prognoses, and approximately onethird of patients died from mass effect resulting from tumour spread within the posterior fossa. [25][26][27] During surgical exploration of Lhermitte-Duclos cerebellar tumours, the margin with normal cerebellar tissue is often not clearly distinct, 28,29 so here we present a case of surgically treated dysplastic cerebellar gangliocytoma and discuss radical surgical resection of the disease. 5 Subsequently, Lhermitte-Duclos disease has become a surgically treatable and even curable condition.…”