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Therapie von primären zerebralen Lymphomen

Treatment of primary cerebral lymphomas

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Zusammenfassung

Bei primär zerebralen Lymphomen (PZNSL) handelt es sich um eine seltene Entität innerhalb der Gruppe der Non-Hodgkin-Lymphome. Die Anamnese ist in der Regel kurz und geht häufig mit neurologischen Ausfällen und Persönlichkeitsveränderungen einher. Goldstandard in der Diagnostik sind die MRT sowie die stereotaktische Biopsie zur Histologiegewinnung. PZNSL reagieren in der Regel sehr sensibel auf Chemotherapie, bereits unter Steroiden können in einem substanziellen Anteil Remissionen erzielt werden. Bei der Wahl der Zytostatika muss der Überwindung der Blut-Hirn-Schranke Rechnung getragen werden. Hoch dosiertes Methotrexat (HD-MTX) ist das effektivste Zytostatikum und obligat in jeder Therapie von PZNSL, die Kombination von hoch dosiertem AraC (HD-AraC) mit HD-MTX ist der Therapie mit HD-MTX allein klar überlegen. Bei jüngeren Patienten kann mittels intensiver Chemotherapieprotokolle bis hin zur autologen Stammzelltransplantation nach hoch dosiertem Carmustin (BCNU) und Thiotepa bei einem substanziellen Teil der Patienten eine Kuration erreicht werden. Für ältere Patienten kann mittels kombinierter (Immun-) Chemotherapieprotokolle ein Langzeitüberleben von bis zu 30% erzielt werden. Aufgrund der Seltenheit der Erkrankung sollte die Therapie möglichst innerhalb klinischer Studien erfolgen.

Abstract

Primary central nervous system lymphomas (PCNSL) are a rare entity within the non-Hodgkins lymphomas. With a short history the clinical presentation frequently occurs with neurologic deficits and/or neuropsychiatric symptoms. Magnetic resonance imaging (MRI) and stereotactic biopsy are required for a definitive diagnosis. PCNSLs are chemosensitive and remission can occur with corticosteroids. The permeability of the blood-brain barrier must be taken into account in the choice of cytostatics. High-dose methotrexate (HD-MTX) is the most effective drug for PCNSL and the combination with high-dose AraC (HD-AraC) has been shown to be more effective than HD-MTX alone. In younger patients intensive treatment protocols as well as high-dose BCNU and thiotepa followed by autologous stem cell transplantation leads to a cure in a substantial proportion of patients. Combined immuno chemotherapy protocols lead to overall survival rates up to 30% in elderly patients. Due to the rarity of the disease patients should be treated according to clinical trial protocols.

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Authors and Affiliations

  1. Medizinische Klinik, Abteilung Hämatologie/Onkologie, Universitätsklinik Freiburg, Hugstetter Straße 55, 79106, Freiburg, Deutschland

    G. Illerhaus & J. Finke

  2. Medizinische Klinik III, Charité – Universitätsmedizin Berlin, Campus Benjamin Franklin, Berlin, Deutschland

    A. Korfel

  3. Neurologische Klinik am Knappschaftskrankenhaus Bochum-Langendreer, Klinikum der Ruhr-Universität Bochum, Bochum, Deutschland

    U. Schlegel

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  1. G. Illerhaus
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  2. J. Finke
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  3. A. Korfel
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  4. U. Schlegel
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Correspondence to G. Illerhaus.

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Illerhaus, G., Finke, J., Korfel, A. et al. Therapie von primären zerebralen Lymphomen. Onkologe 17, 308–317 (2011). https://doi.org/10.1007/s00761-010-1963-y

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