Gangliogliomas are uncommon CNS tumours which account for around 2% (from 0.4-3.8%) of all primary intracranial tumours; and up to 10% of primary cerebral tumours in children. They are, however, the most frequent of the neuronal-glial CNS neoplasms 6.
They are of low grade (WHO Grade I or II). Typical occurrence is in the temporal lobes, although they have been described in all parts of the central nervous system.
Children and young patients are usually affected, and no gender predominance is recognised.
The most common presentation is with temporal lobe epilepsy, presumably due to the temporal lobes being a favoured location.
Gangliogliomas are composed of two cell populations:
- ganglion cells (large mature neuronal elements): ganglio-
- neoplastic glial elements (primarily astrocytic): -glioma
It is the grade of the glial component that determines biological behaviour. Dedifferentiation into high grade tumours does occasionally occur, and it is usually the glial component (into a GBM). Only rarely is it the neuronal component (into a neuroblastoma).
Neuronal origin is demonstrated by positivity to neuronal markers such as:
- neuronal specific enolase
May also show positivity to the GFAP marker.
There is predilection towards the temporal lobes 6.
Imaging findings mirror the various patterns of growth which these tumours may demonstrate, and unfortunately their appearance is very variable. Partially cystic mass with an enhancing mural nodule is seen in 35-55% of cases. They may also simply present as a solid mass expanding the overlying gyrus. An infiltrating mass is uncommon and may reflect higher grade.
Findings are of a mass which is often non specific. General features include:
- iso- or hypodense
- frequently calcified ~35%
- bony remodeling or thinning can indicate the slow growing nature of the tumour
- enhancement is seen in approximately 50% of cases (involving the solid non-calcified component)
Reported signal characteristics include:
- T1: iso to hypo intense
- C+ (Gd): variable contrast enhancement
- T2: hyperintense solid component, with variable signal in the cystic component depending on amount of proteinaceous material or presence of blood products; peritumoral FLAIR/T2 oedema is distinctly uncommon
- GE/SWI: calcified areas will show blooming
Treatment and prognosis
Local resection is the treatment of choice and determines prognosis. In the brain where a reasonable resection margin can be achieved, prognosis is good. In the spinal cord where this is not possible without devastating deficits local recurrence is very common.
If only incomplete resection is achievable, or tumour recurrence occurs then radiotherapy may be of some benefit.
The differential diagnosis is very broad and should be restricted by location.
If in the temporal lobe consider:
- pleomorphic xanthoastrocytoma (PXA)
- pilocytic astrocytoma
- dysembryoplastic neuroepithelial tumour (DNET)
- cystic metastases
- papillary glioneuronal tumour
If in the spinal cord consider:
For cortical tumours consider:
- 1. Kwon JW, Kim IO, Cheon JE et-al. Cerebellopontine angle ganglioglioma: MR findings. AJNR Am J Neuroradiol. 2001;22 (7): 1377-9. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 2. Patel U, Pinto RS, Miller DC et-al. MR of spinal cord ganglioglioma. AJNR Am J Neuroradiol. 1998;19 (5): 879-87. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 3. Provenzale JM, Ali U, Barboriak DP et-al. Comparison of patient age with MR imaging features of gangliogliomas. AJR Am J Roentgenol. 2000;174 (3): 859-62. AJR Am J Roentgenol (full text) - Pubmed citation
- 4. Castillo M. Gangliogliomas: ubiquitous or not? AJNR Am J Neuroradiol. 1998;19 (5): 807-9. AJNR Am J Neuroradiol (citation) - Pubmed citation
- 5. 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
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