Gliomatosis cerebri is a diffusely infiltrative glial tumour that involves at least three lobes by definition 5. There often is an important discordance between clinical and radiological findings, as it may be clinically silent while it appears as a very extensive process radiologically.
Peak incidence is at 20-40 years of age.
The tumour may be primary GC (de novo) or secondary, the last one as a result from the spreading of a focal glioma 5.
Can be normal because lesions often isodense to normal brain parenchyma. There is relative lack of mass effect and distortion. There may be an ill defined asymmetry or subtle hypoattenuation to the involved brain parenchyma.
Mass effect and enhancement are minimal. There is loss of GM/WM differenciation and diffuse gyral thickening.
Diffuse T1 and T2 prolongation throughout both white and grey matter.
- T1: iso to hypointense to grey matter 1
- T2: hyperintense to grey matter 1
- MR spectroscopy: elevated Cho:Crn and Cho:NAA ratios 2
Treatment and prognosis
The condition carries a poor prognosis with an average survival of ≈ 50% at 1 year and 25 % at 3 years. GBM/HAA may occur a few years later.
Surgery is not a suitable option due the diffuse nature of this tumour.
Radiotherapy is an option, however the large field usually required in these lesions increases the risk of severe toxicity 5.
General imaging differential considerations include:
- progressive multifocal leukoencephalopathy (in immunocompromised patient): no mass effect
- WHO classification of CNS tumours
- diffuse astrocytic tumours
- diffuse astrocytoma grading
- low grade astrocytoma
- anaplastic astrocytoma
- glioma treatment response assessment in clinical trials
- O6-Methylguanine-DNA methyltransferase (MGMT)
- giant cell glioblastoma
- gliomatosis cerebri
- radiation-induced gliomas
- localised astrocytic tumours
- specific locations
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