Around 300 to 400 people, mainly adults, are diagnosed with primary CNS lymphoma (PZNSL for short) every year in Germany. This is an accumulation of malignantly altered immune cells ("lymphocytes") in the central nervous system (CNS).
Here you will find further information as well as selected specialists and centers for CNS lymphomas.
The nerve structures in the brain and spinal cord are known as the central nervous system (CNS). They are responsible for central stimulus processing in humans.
CNS lymphoma is a cancer that occurs
can occur. Occasionally, the eyes are also affected in the form of retinal, uveal or vitreous infiltration. However, with a frequency of only around ten percent, this is the rarest form of CNS lymphoma.
CNS lymphomas, which are classified as non-Hodgkin's lymphomas, are divided into primary and secondary CNS lymphomas.
If tumor cells are located exclusively in the central nervous system at the time of initial diagnosis, this is referred to as primary CNS lymphoma. Secondary CNS lymphomas are metastases in the central nervous system. Metastases are metastases of a tumor that initially developed in other parts of the body.
Diffuse large B-cell lymphoma is the most common variant, accounting for over 95 percent of cases. It is considered "highly malignant", i.e. particularly aggressive and fast-growing. Other B-cell or T-cell lymphomas, on the other hand, are very rarely diagnosed in the central nervous system.
How lymphocytes degenerate and form tumors is still largely unknown. However, researchers assume that the cause could lie in the development of certain proteins in the central nervous system. These could possibly trigger an uncontrolled immune response that promotes the proliferation of lymphoma cells.
The risk factors for CNS lymphoma have also not yet been fully researched beyond doubt. However, the majority of people affected by lymphoma of the central nervous system are adults. A good half of patients are already 60 years old or older at the time of initial diagnosis. Men are affected slightly more frequently than women. There is no evidence of a familial clustering.
The majority of patients with a CNS tumor did not suffer from an immune deficiency before the disease. Nevertheless, this is considered one of the greatest risk factors: suppression of the body's own immune system (immunosuppression) can lead to the formation of a lymphoma in the central nervous system.
It is irrelevant whether
The symptoms of CNS lymphoma can vary greatly. They depend on the size of the tumor and its exact location in the central nervous system.
The following symptoms are most commonly associated with CNS lymphoma:
As a rule, the initial diagnosis of CNS lymphoma is made relatively quickly. This is mainly because the tumor grows relatively quickly and the signs of the disease progress accordingly.
However, some of the symptoms are also typical of other diseases of the central nervous system. If the symptoms are not particularly pronounced and rather unspecific, a diagnosis can sometimes take a little longer.
If a brain disease is suspected, an examination of the head is usually carried out using computer tomography(CT). If the doctor discovers a tumor, it is important to find out as quickly as possible what type it is and how advanced the disease is.
This is followed by
Magnetic resonance imaging(MRI) provides the most conclusive results. However, the images do not allow the type of brain tumor to be identified beyond doubt. A tissue sample of the lymphoma is required for a reliable diagnosis.
A CNS lymphoma in the brain can be clearly visualized using magnetic resonance imaging © Chinnapong | AdobeStock
The tissue sample is taken by means of a stereotactic biopsy using a hollow needle through a tiny drill hole in the skull. This procedure is the most common method today.
Once a CNS lymphoma has been diagnosed, the doctors look for other lymphoma foci in the body. This is aided by
Finally, it could also be a secondary CNS lymphoma, i.e. the metastasis of another tumor.
Without any treatment, a CNS lymphoma would lead to death within a short time. Rapid action is therefore required.
However, complete surgical removal, as is often performed for other brain tumors, is not effective for CNS lymphoma. A combination of chemotherapy and radiotherapy or chemotherapy alone is more promising in the long term.
Depending on the age of the patient, both the effects and side effects of the respective form of therapy vary. For this reason, an individually tailored treatment path must always be chosen. A distinction is made between
The chemotherapeutic agent methotrexate is most commonly used in induction therapy. It is also popular because it can be administered to patients of all ages. The substances cytarabine, ifosfamide and thiotepa are also effective.
In addition to chemotherapy, doctors often opt for antibody therapy (known as chemoimmunotherapy in combination). The active substance rituximab binds to the surface structures of the tumor cells and triggers their destruction.
In consolidation therapy
can be used. However, radiotherapy can have neurological consequences such as impaired memory or quality of life. For this reason, alternatives should be preferred if possible.
HDT-ASCT in particular has often led to very good long-term results. In this procedure, stem cells are removed from the patient before chemotherapy and then reintroduced.
Specialists in haematology and oncology at established treatment centers are the right point of contact for patients with CNS lymphoma.
The Malignant Lymphoma Competence Network can help you find experts. Here you will find hospitals and specialist practices with many years of experience in the treatment of CNS lymphoma.
The self-help organizations for leukaemia and lymphoma sufferers are organized in the Bundesverband Deutsche Leukämie- & Lymphom-Hilfe e.V. (German Leukaemia & Lymphoma Aid Association ). Here you can search for your nearest self-help group. There is also a wide range of brochures, literature and event information available.
CNS lymphoma is considered to be particularly aggressive. Nevertheless, the disease can be suppressed for several years with a therapy that is optimally tailored to the patient.
In younger patients whose immune system is functioning well, a complete cure may even be possible.
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