What is a glioblastoma?

A glioblastoma is a malignant brain tumor that is very aggressive and grows quickly. Glioblastoma can develop and quickly spread to healthy brain tissue within just a few weeks or months. Glioblastoma is the most common malignant brain techsupportreviews tumor in adults - and at the same time the most dangerous of the brain tumors (medical gliomas).

Glioblastoma has its origin in the glial cells - these are supporting cells in the brain that not only form a framework for nerve cells, but are also involved in various processes such as oxygen transport or the maintenance of the brain's state of equilibrium (homeostasis). These degenerate and divide and then multiply unchecked. The tumor cells of the glioblastoma come from the so-called astrocytes. This is why experts also refer to glioblastomas as grade 4 astrocytomas. With a share of more than 60 percent, astrocytomas are the most common gliomas.

 There are two groups:

• Astrocytomas that grow diffusely into the brain tissue: diffuse astrocytoma (WHO grade 2), anaplastic astrocytoma (WHO grade 2), and glioblastoma (WHO grade 4). They occur more frequently in adults and have a rather unfavorable prognosis.
• Astrocytomas , the growth of which in the brain tissue can be better defined. The most important is pilocytic astrocytoma (WHO grade 1). These astrocytomas occur mainly in children and young adults, grow slowly and rarely degenerate. Cure is possible if doctors completely remove the tumor through surgery.

In technical terms, the glioblastoma is also called glioblastoma multiforme. The name comes from the facts that it shows a very "colorful" and diverse appearance under the microscope: Pathologists recognize cavities, dead tissue and hemorrhages. Most people between the ages of 45 and 70 develop this type of cancer. Most glioblastomas develop anew from glial cells. Doctors refer to these as primary glioblastomas . Sometimes the glioblastoma also forms from an already existing diffuse or anaplastic astrocytoma - then doctors speak of secondary glioblastomas .

Glioblastoma: very dangerous, but comparatively rare

The World Health Organization (WHO) divide gliomas into different grades depending on how dangerous they are:

• Grade 1 (benign)
• Grade 2 (rather benign)
• Grade 3 (already malignant)
• Grade 4 (malicious and aggressive)

Because glioblastoma is so aggressive, the WHO classification assigns glioblastoma to grade 4 of brain tumors. This is the most severe degree a brain tumor can reach.

Compared to other cancers such as breast cancer , prostate cancer , colon cancer or lung cancer , however, gliomas are rather rare. Every year only about five to six out of 100,000 German citizens contract it. Men are more often affected than women (ratio 6: 4) It is unclear why this is the case. Depends on the cell type from which the glioma originates, astrocytomas, oligodendrogliomas and a few other forms can be distinguished. Doctors diagnose the most malignant form in about half of patients - glioblastoma 4.

Glioblastoma: life expectancy is often short

The prognosis for glioblastoma is rather poor. The reason is that this brain tumor is very aggressive and spreads quickly into healthy brain tissue. Many patients therefore do not survive a glioblastoma very long. Depending on the variant of the tumor , patients have an average life expectancy of at best more than 15, at worst just under a year. Only about 14 percent of patients with glioblastoma survive the first two years after diagnosis. After five years, only about five percent of patients are still alive.

According to the Society of Epidemiological Cancer Registers in Germany, both new cases (men: + 1.5% / year, women: + 1.3% / year) and deaths (men: + 1.5% / year, women: + 0.6% / year) increased somewhat in the past ten years. Experts suspect that the reason is the change in the population structure, i.e. that there are more and more people in old age and life expectancy has generally increased.

Doctors can treat the brain tumor with surgery, chemotherapy, and radiation therapy and keep it in check for some time. But it always depends on how the therapy works and how the patient tolerates it. In the case of severe side effects, the treatment may do more harm than good because it severely reduces the patient's quality of life.

As a rule, doctors cannot cure glioblastoma. Because even in spite of initially successful therapy, the brain tumor returns quickly in many cases.

Glioblastoma: The course is usually rapid

Glioblastoma spreads rapidly in the brain. However, brain tumors very rarely form cancerous deposits in other organs. In glioblastoma, metastases in other organs such as the lungs, liver or bones are rare. Although this brain tumor is so malignant, it is usually confined to the central nervous system (CNS). In the end-stage glioblastoma, however, the tumor cells can spread to other locations in the CNS via the cerebrospinal fluid. Doctors can then only alleviate the symptoms and try to maintain the patient's quality of life as well as possible.

Caution: A brain tumor is not to be confused with brain metastases. The difference: in the latter, the original tumor is in a different location and from there spreads its cancer cells, the metastases, into the brain. These daughter tumors often develop at an advanced stage in other types of cancer such as breast, lung or black skin cancer .

Glioblastoma: Symptoms differ depending on where they originate

Glioblastoma symptoms depend on which region of the brain the malignant tumor developed in. Each area of ​​the brain is responsible for different tasks. If the functions in these regions fail or if they are impaired by the tumor, different signs develop. Symptoms of a glioblastoma can include:

• Headaches that come on, persist and get worse - especially at night or early in the morning; they usually improve during the day; the reason is that the tumor increases intracranial pressure
• Dizziness from increased pressure in the brain
• Nausea and vomitings due to increased pressure in the brain, especially in the morning or on an empty stomach
• Seizures and convulsions
• Neurological symptoms : paralysis, impaired vision, speech, movement or coordination
• Changes in personality and character : e.g. B. easy irritability, disorientation

Because glioblastomas grow quickly, symptoms usually develop within a few weeks or months. Always consult your doctor quickly if you experience such warning signs.

 Glioblastoma: cause is unclear

The cause of the glioblastoma is still largely unclear. It is known that certain supporting cells, the astrocytes, degenerate (mutate) in the brain. The tumor cells then divide and multiply rapidly. Why these mutations take place is still unknown. Experts assume that they happen by chance, for example due to errors in cell division. A glioblastoma can also result from an existing grade 2 or 3 astrocytoma. However, this happens less often.

The increased exposure to ionizing radiation seems to play a role as a risk factor. People with cancer often receive radiation therapy in which they are exposed to this radiation in harmful doses. So treating another (cancer) tumor can cause glioblastoma. In addition, some very rare hereditary diseases increase the likelihood of a glioblastoma, for example:

• Neurofibromatosis type 1 (changes on gene 17) and type 2 (changes on gene 22). Type 1 is significantly more common than type 2.
• Von Hippel-Lindau syndrome : the changes are on chromosome 3
• Li Fraumeni syndrome : Children and adolescents develop a wide variety of tumors at an early age

Even if the genes are involved, one cannot say that glioblastoma is hereditary - at least not directly.

Glioblastoma: Therapy slows tumor growth

When it comes to glioblastoma therapy, doctors use various strategies, which they usually combine with one another. Very often this does not cure the glioblastoma, but slows its progression. During treatment, it is not only important to gain life, but also to maintain the quality of life for as long as possible.

Doctors use these treatments for glioblastoma:

• Operation : Neurosurgeons try to remove the tumor as completely as possible during an operation. In many cases, however, this does not succeed because the glioblastoma quickly grows into healthy tissue and spreads. The operation is a very difficult procedure that only an experienced neurosurgeon should perform. Subsequent neurological disorders and failures are usually unavoidable because the brain is a very complex structure.
• Radiation therapy (radiotherapy): This glioblastoma therapy usually follows the operation. Radiologists try to use radiation to fight cancer cells that have remained in the brain. The rays damage the genetic material of the tumor cells. Because they cannot repair the damage, they die. This can often delay the progression of the glioblastoma.
• Chemotherapy : Patients with glioblastoma are given cell-killing agents ( cytostatics ) that work throughout the body. Doctors use the active ingredient temozolomide alone or in combination with the cytostatic agent CCNU.
• Antibody Bevacizumab : The active ingredient prevents the formation of new blood vessels and cuts off the tumor from the oxygen and nutrient supply. This is how doctors want to "starve out" cancer. Bevacizumab belongs to the group of drugs called angiogenesis inhibitors.
• Psycho-oncology : Glioblastomas are associated with an unfavorable prognosis. There are therefore various psycho-oncological offers for psychological support for patients. These are psychological support offers especially for cancer patients.

There are also effective drugs that can alleviate the symptoms of cancer and the side effects of the therapies.

MGMT status in glioblastoma

In elderly patients with glioblastoma, doctors determine a special characteristic in order to be able to better assess the effectiveness of chemotherapy: the methylation status of the MGMT promoter . Patients with such a chemical tag (methyl group) on this gene particularly benefit from chemotherapy. They can't make a special enzyme that helps repair cancer cells. The MGMT status can thus serve as a biomarker of whether a patient benefits from chemotherapy. In this case, they will receive temozolomide alone and possibly also radiation therapy. For patients with tumors with no or unknown MGMT status, radiation therapy alone is the standard.

Treat relapsed glioblastoma

Glioblastomas tend to return quickly - usually within a year and despite initially successful therapy. Doctors refer to a relapse as a relapse. If a glioblastoma relapses, they rely on a new operation, radiation and chemotherapy, possibly with a modified schedule of cytostatic drugs.

Researchers are currently working in studies on other options for glioblastoma therapy to stop tumor growth. One example is the combination treatment of the two antibodies with the abbreviations anti-EGFL7 and anti-VEGF with the cytostatic drug temozolomide. The antibodies belong to the so-called angiogenesis inhibitors . These drugs prevent the formation of new blood vessels, which the tumor needs to supply oxygen and nutrients.

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