Gliomatosis Cerebri Symptoms & Causes

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What causes gliomatosis cerebri?

There are no known factors or conditions that would make your child more or less likely to develop gliomatosis cerebri.

What are the symptoms of gliomatosis cerebri?

Symptoms might come on slowly and subtly, or they might appear more abruptly. Each child may experience symptoms differently, and the most common include:

  • headache and lethargy
  • symptoms of increased pressure within the brain, including:
    • headache (generally upon awakening in the morning)
    • vomiting
    • seizures - can occur with high-grade gliomas in the cerebrum but are less common with low-grade lesions (any abnormal tissue).
  • localized symptoms - these tumors invade normal tissue as they grow, and produce symptoms based on their location. These could include:
  • weakness and other motor dysfunction
  • neuroendocrine (hormones affecting the nervous system) abnormalities
  • changes in behavior or thought processes

The symptoms gliomatosis cerebri may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.

How is gliomatosis cerebri classified?

The World Health Organization classification scheme includes 4 grades of glioma.

  • grade I (benign)
  • grade II (atypical)
  • grade III (anaplastic – refers to lack of structure in the cell)
  • grade IV (sarcomatous – the most serious kind of tumor)

However, since only a fraction of gliomatosis cerebri tumors are biopsied, it can be difficult to conclusively assign a grade to them.

That said, they usually progress like grade IV glioblastoma multiforme tumors. These are the most aggressive kind of astrocytic tumor, and they usually have the following characteristics:

  • hypercellularity (increased number of cells)
  • cytologic and nuclear atypia (abnormal cells and nuclei)
  • mitoses (the cells reproduce rapidly)
  • necrosis (the cells die quickly)
  • vascular endothelial proliferation (increased growth of blood vessels)

These tumors are aggressive, and will invade normal brain tissue. They are also likely to spread outside the central nervous system to other parts of the body.

What is the latest research on gliomatosis cerebri?

A variety of chemotherapeutic regimens have been evaluated in the treatment of newly diagnosed high-grade gliomas.

  • While studies in adults have suggested that procarbazine, vincristine and CCNU (PVC) produce modest responses in grade III gliomas, this has not been demonstrated for pediatric cases or for grade IV tumors.
  • Several other regimens have also produced responses, but none has improved survival.
  • Increased doses of chemotherapy in the setting of autologous bone marrow transplant have also not produced notable advantage.

Boston Children's Hospital and Dana-Farber Cancer Institute are conducting many research studies that will help clinicians better understand and treat gliomatosis cerebri. For more information on our current research initiatives, see the Brain Tumor Program.

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