CNS lymphoma

The CNS lymphoma is a rare form of cancer of the lymph tissue in the central nervous system (CNS). A distinction is made between primary CNS lymphoma (PZNSL), which occurs first in the CNS, and secondary CNS lymphomas, which arise as metastases from lymphomas in other parts of the body.

Epidemiology

Since the AIDS epidemic in the 1980s, the incidence of CNS lymphoma has risen sharply, as there is a strong association between PZNSL and immunosuppression . In AIDS patients, CNS lymphoma is the most common brain tumor of all today . However, more immunocompetent people are also affected.

In 5–29% of all systemic non-Hodgkin lymphomas there is a settlement in the brain, that is, a secondary CNS lymphoma. However, metastasis to the brain usually only occurs in an advanced and disseminated disease.

Pathophysiology

There is still no explanation for the development of CNS lymphoma, but some risk factors have been identified:

• Immunosuppression
• Diseases of the rheumatic form circle
• EBV infection

Histogenetic studies provide evidence of mutations of the proto-oncogene BCL-6 , which, especially in non- AIDS -associated CNS - lymphoma may play a role. Both the EBV virus and the HHV-8 virus could play a role in the pathogenesis of both immunocompromised and immunocompetent persons .

A widespread hypothesis is that the PZNSL can develop more easily because the brain is an immunological "sanctuary". In other parts of the body, the degenerated lymphocytes would be destroyed by the immune system , but they have no access to the CNS .

clinic

In immunocompetent kicking CNS - lymphoma primarily on the 5th decade of life. In the case of immunosuppressed, however, already in the 2nd and 3rd decades of life. Most often there is diffuse and infiltrative growth with supratentorial localization that progresses rapidly. In a few weeks to months, symptoms such as personality changes, cerebellar symptoms and asymptomatic meningiosis occur. The clinical picture of encephalopathy is often found in immunosuppressed persons .

Diagnosis

Imaging in particular plays a major role. In up to 50% there is a multifocal infestation, especially in the frontal and parietal lobes .

• Angiography : avascular structure
• CT : iso- or hyperdense, close to the ventricle, stores contrast medium
• MRI
• Lumbar puncture
• Brain biopsy : method of choice to confirm the diagnosis
• prompt and often complete regression to steroids (steroids should be avoided before a biopsy as they obscure the diagnosis)

therapy

The focus is on brain-penetrating chemotherapeutic agents (e.g. methotrexate ) and radiation therapy . Surgical removal is usually not indicated due to the initially very good response to radiation and chemotherapy, which often results in complete temporary regression. For unifocal primary CNS lymphomas, however, this guiding principle was called into question by a 2012 analysis.

The therapy often responds well, especially in immunocompetent people.

literature

• S1 guideline for primary CNS lymphomas (PZNSL) of the German Society for Neurology. In: AWMF online (as of 2012)
• W. Hiddemann et al .: Oncology. Part 2: Special Part. Springer, Heidelberg 2010, ISBN 978-3-540-79724-1 , pp. 1569ff.

Individual evidence

1. ↑ Primary CNS lymphomas (PZNSL). German Society for Neurology , January 8, 2015, accessed January 1, 2017 . 

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !