Gliomatosis Cerebri

What is Gliomatosis Cerebri?

Gliomatosis cerebri is a highly aggressive, rare form of malignant astrocytic tumour. It is most commonly present as a diffusely infiltrating glial tumour of the cerebral cortex. It is kind of like a thin film spread all over the brain, with the possibility of larger tumours springing up from the film in localized areas. Glial cells are like the connective tissue of the brain and insulate the nerves that are in the brain, so this is why the tumour spreads in a spider-web like pattern. There are no known causes or conditions that predispose an individual to developing this disease. The prognosis remains poor, with an average survival of 12 months after diagnosis.

What Causes Gliomatosis Cerebri?

Gliomatosis cerebri of the cerebral hemispheres can produce non-specific symptoms as a result of increased pressure within the head, as well as more localizing symptoms as a function of specific tumour location, rate of growth and associated inflammation.

The duration of symptoms is variable from a slow, insidious onset to more abrupt presentations. The following are the most common symptoms of gliomatosis cerebri, however each child may experience symptoms differently. Common symptoms may include:

  • headache and lethargy
  • symptoms of increased pressure within the brain

There symptoms include:

  • headache (generally upon awakening in the morning)
  • vomiting
  • seizures – can occur with supratentorial high grade gliomas, but do so less commonly than with low grade lesions
  • localized symptoms – these tumours infiltrate normal structures as they grow and produce symptoms as a function of their location

These can include:

  • weakness and other motor dysfunction
  • neuroendocrine abnormalities
  • changes in behaviour or thought processes

Gliomas are heterogeneous tumours that are classified according to their most aggressive appearing elements. The World Health Organization classification scheme includes 4 grades of glioma. The pathological grade of gliomatosis cerebri is not always established, because only a fraction of these tumours is biopsied. Their clinical course is most consistent with an aggressive form of astrocytic tumour. These tumours are characterized as being of astrocytic origin but having increased numbers of cells (hypercellularity), abnormal cells and nuclei (cytologic and nuclear atypia), increased proliferation of cells (mitoses), increased cell death (necrosis) and increased growth of blood vessels (vascular endothelial proliferation). These are aggressive tumours that infiltrate adjacent normal brain tissue and have a significant tendency to spread outside of the central nervous system.

A diagnosis of gliomatosis cerebri is most commonly made from characteristic radiologic studies. They can lack a distinct primary location and their diffusely infiltrating growth makes biopsy a high-risk procedure. Biopsies have been performed when primary masses are identified or when the symptoms and other tests do not seem typical for gliomatosis cerebri.

Treatment may include:

  • radiation therapy – the primary therapy for newly diagnosed gliomatosis cerebri, radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumours

There is no surgical option in the treatment of these tumours. Experimental chemotherapy delivered concurrent to radiation therapy is actively being investigated in the treatment of diffuse gliomatosis cerebri. Several trials evaluating new agents such as thalidomide and temazolamide are either underway or recently completed. In addition, there are trials evaluating whether improved response to traditional chemotherapeutic agents may be obtained via novel delivery systems to more directly introduce the drugs into the brain. Data on the efficacy of these regimens should be available soon. To date, no chemotherapy regimen has been demonstrated to increase survival in gliomatosis cerebri.

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