Gangliocytoma

Gangliocytomas are rare indolent CNS tumours (WHO grade I), 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 tumours are defined by the presence of displaced ganglion cells (large mature neurons that show cytological or architectural abnormalities).

On imaging these tumours are usually characterized by cortical solid lesions with little associated mass effect and minimal or no surrounding vasogenic oedema. Calcification and cyst formation can occur, and contrast enhancement is generally present.

Gangliocytomas should not be confused with dysplastic cerebellar gangliocytoma of the cerebellum, better known as Lhermitte-Duclos disease.

Gangliogliomas account for 0.1-0.5% of all brain tumours. They tend to be diagnosed in children and young adults 2.

No clinical differences between gangliogliomas and gangliocytomas are recognised. Tumours in the cerebral cortex present most commonly with epilepsy 4.

Gangliocytomas may arise anywhere within the neuroaxis 4.

Microscopic appearance

The tumour is composed of abnormal large mature neurones, usually with a multipolar morphology 5. Some neurones are binucleated 5. The key feature in distinguishing ganglicytomas from gangliogliomas is identifying a lack of neoplastic glial cells.

Immunophenotype

Appearances of gangliocytomas are indistinguishable form gangliogliomas 5.

CT

Gangliocytomas typically appears hyperattenuating on non contrast imaging. They usually have little associated mass effect and minimal or no surrounding vasogenic oedema. Calcification and cyst formation can occur.

MRI
  • 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

These tumours tend to grow slowly and do not undergo anaplastic change. Resection is curative.


Malformations of the central nervous system
Share Article

Article Information

rID: 5501
Synonyms or Alternate Spellings:

Support Radiopaedia and see fewer ads

  • Drag
    Case 1: on MRI T2
    Drag here to reorder.
  • Drag
    C+ CT
    Case 1: on CT
    Drag here to reorder.
  • Drag
    Case 3: right frontal lobe
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.