Gangliocytoma

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===Definition===
 
===Definition===
 
Gangliocytoma is a well-differentiated, slowly growing neuroepithelial tumor composed of neoplastic mature ganglion cells. Gangliocytoma is considered a WHO Grade I tumor.
 
Gangliocytoma is a well-differentiated, slowly growing neuroepithelial tumor composed of neoplastic mature ganglion cells. Gangliocytoma is considered a WHO Grade I tumor.

Revision as of 18:25, 10 July 2008

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Contents

Definition

Gangliocytoma is a well-differentiated, slowly growing neuroepithelial tumor composed of neoplastic mature ganglion cells. Gangliocytoma is considered a WHO Grade I tumor.

Epidemiology

Gangliocytoma represents less than 1% of all brain tumors. The peak incidence is in children and young adults between age 8.5 and 25 years, with a mean age of occurrence at 9.5 years. There is a slight female predominance (1.1:1).

Localization

Gangliocytoma may occur throughout the CNS, but are most commonly found in the temporal lobes.

Clinical presentation

Symptoms are site-specific, but are stereotypically long-standing.

Imaging

MRI demonstrates a well-demarcated lesion with variable contrast enhancement. Scalloping of the calvarium may be seen adjacent to superficially located tumors.

Macroscopy

Gangliocytoma are solid or cystic tumors that are well delineated from the surrounding normal brain. Calcifications may be present.

Histopathology

Gangliocytoma are composed of irregular groups of large, multipolar neurons that often show dysplastic features, harbored within a stroma of non-neoplastic glial elements and a network of reticulin fibers. Perivascular lymphocytic infiltration is typical.

Molecular genetics

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos) occurs in the setting of Cowden disease, which is caused by a germline PTEN mutation. The molecular genetics of sporadic gangliocytoma have not been elucidated.

Prognosis

Gangliocytoma is a surgically curable disease.

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