What it is?

Childhood giomatosis cerebri is a rare and incurable disease of which little is known.

The incidence of the disease is unknown, though one paediatric hospital has suggested that it accounts for 3% of all brain tumours.

Gliomatosis cerebri is a highly aggressive, malignant brain tumour. It is a disease of the glial cells, usually the astyocytes (glial cells in the nervous system whose long protrusions give them a star-like shape). Its main characteristic is the rapid, diffuse spread of the tumour, which means that its growth infiltrates the brain without defined borders, unlike other brain tumours. As it grows, it is capable of reaching any part of the brain.

It affects more than two cerebral lobes, often bilaterally, and usually reaches infratentorial structures (the cerebellum and brain stem), and even the spinal cord. The infiltration leads to a thickening of anatomical structures, affecting the white matter, whose principal function is to transmit nerve impulses from the neurones and deep brain nuclei (such as the thalamus, which helps to control movement). The spread can easily cross the corpus callosum, the structure which connects the right and left hemispheres of the brain, coordinating their functioning.

In contrast to other malignant astrocytic tumours, even in very advanced stages, the disease often takes the form of a diffuse infiltration, as opposed to a well-defined tumour. This is the most common form of gliomatosis cerebri, or type 1, whilst patients with an associated tumour in addition to this diffuse infiltration form part of the rarer group 2.