Citation, DOI, disclosures and article data
At the time the article was created Frank Gaillard had no recorded disclosures.View Frank Gaillard's current disclosures
At the time the article was last revised Frank Gaillard had no recorded disclosures.View Frank Gaillard's current disclosures
Gangliocytomas are rare indolent CNS tumors (WHO grade 1), primarily encountered in children, and frequently discovered as the cause of epilepsy. They differ from gangliogliomas by the absence of neoplastic glial cells, although both tumors are defined by the presence of displaced ganglion cells (large mature neurons that show cytological or architectural abnormalities).
On imaging, these tumors are usually characterized by cortical solid lesions with little associated mass effect and minimal or no surrounding vasogenic edema. Calcification and cyst formation can occur, and contrast enhancement is generally present.
On this page:
Gangliocytomas should not be confused with dysplastic cerebellar gangliocytoma of the cerebellum, better known as Lhermitte-Duclos disease.
Gangliocytomas account for 0.1-0.5% of all brain tumors. They tend to be diagnosed in children and young adults 2.
No clinical differences between gangliogliomas and gangliocytomas are recognized. Tumors in the cerebral cortex present most commonly with epilepsy 4.
Gangliocytomas may arise anywhere within the neuroaxis 4.
The tumor is composed of abnormal large mature neurons, usually with a multipolar morphology 5. Some neurons are binucleated 5. The key feature in distinguishing gangliocytomas from gangliogliomas is identifying a lack of neoplastic glial cells.
- synaptophysin: positive
- neurofilament: positive
- chromogranin-A: positive
- MAP2: positive
- GFAP: negative
Appearances of gangliocytomas are indistinguishable from gangliogliomas 5.
Gangliocytomas typically appears hyperattenuating on non-contrast imaging. They usually only have little associated mass effect and minimal or no surrounding vasogenic edema. Calcification and cyst formation can occur.
- T1: solid components typically hypointense
- T2: solid components are typically mildly hypointense 2; cystic areas are hyperintense; calcification if present can be hypointense
- T1 C+ (Gd): solid components enhance
Treatment and prognosis
These tumors tend to grow slowly and do not undergo anaplastic change. Resection is curative.
- 1. Altman NR. MR and CT characteristics of gangliocytoma: a rare cause of epilepsy in children. AJNR Am J Neuroradiol. 1988;9 (5): 917-21. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 2. Koeller KK, Henry JM. From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 21 (6): 1533-56. Radiographics (full text) - Pubmed citation
- 3. Shin JH, Lee HK, Khang SK et-al. Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation. Radiographics. 22 (5): 1177-89. Radiographics (full text) - Pubmed citation
- 4. Shorvon SD, Andermann F, Guerrini R. The Causes of Epilepsy. Cambridge University Press. (2011) ISBN:113949578X. Read it at Google Books - Find it at Amazon
- 5. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central Nervous System. 4th Edition Revised" ISBN: 9789283244929