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Gliomatosis cerebri

Dr Henry Knipe and Dr Frank Gaillard et al.

Gliomatosis cerebri is a rare, diffusely infiltrative glial tumour that involves at least three lobes by definition. 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.

Epidemiology

  • peak incidence is 20-40 years of age
  • M:F of 1.5:1 in one sequential series of 54 patients 7

Pathology

The tumour may be primary (de novo) or secondary, with the latter as a result from the spreading of a more focal glioma 5. Gliomatosis cerebri can contain areas of WHO grade 2 or 3 tumours, and rarely grade 4 6,7

Classification

Gliomatosis cerebri can be divided into two types 7:

  • type 1: no discrete mass
  • type 2: discrete mass with further diffuse CNS involvement

Radiographic features

CT

CT 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.

MRI

Mass effect and enhancement are minimal. There is loss of gray-white matter differentiation 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
  • T1C+: typically no or minimal enhancement
  • DWI: usually no restriction
  • MR spectroscopy: 
    • elevated Cho:Crn and Cho:NAA ratios 2
    • marked elevation of myoinositol (mI)
  • perfusion MR:
    • ​low/normal rCBV: correlates with no vascular hyperplasia
PET
  • FDG PET shows marked hypometabolism

Treatment and prognosis

The condition carries a poor prognosis with an average survival of ≈ 50% at 1 year and 25% at 3 years. Transformation into glioblastoma may occur a few years later.

Surgery is not a suitable option due the diffuse nature of this tumour but can be used to treat complications such as hydrocephalus.

Radiation therapy has been shown to increase survival 7, however, the large field usually required in these lesions increases the risk of severe toxicity 5. Chemotherapy is a treatment option, although there is a lack evidence in its use (both positive and negative) 7.

Differential diagnosis

General imaging differential considerations include 6:


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