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Primäre ZNS-Lymphome

Primary CNS lymphomas

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Zusammenfassung

Primäre ZNS-Lymphome (PZNSL) gehören zu den seltenen Gehirntumoren. Sie machen etwa 3% aller primären intrakraniellen Neubildungen aus; das mediane Erkrankungsalter liegt zwischen 60 und 65 Jahren. PZNSL führen häufig zu einem rasch progredienten Psychosyndrom und zeigen sich in der Magnetresonanztomographie (MRT) oft als multifokale oder unifokale, intensiv kontrastmittelaufnehmende Läsionen, häufig in der Nachbarschaft zu den Ventrikeln. Die diagnostische Maßnahme der Wahl ist die stereotaktische Biopsie, die bei mehr als 90% der Patienten ein hoch malignes Non-Hodgkin-Lymphom (NHL) der B-Zell-Reihe vom diffus-großzelligen Typ zeigt. Da weiterhin eine Therapieoptimierung der PZNSL angestrebt wird, sollten Patienten innerhalb klinischer Studien behandelt werden. Bei Patienten unter 60 Jahren ist das Therapieziel kurativ. Jungen Patienten sollte eine Polychemotherapie unter Einschluss von systemischem hochdosiertem Methotrexat (MTX) angeboten werden. Der Stellenwert einer myeloablativen Hochdosischemotherapie mit autologer Stammzelltransplantation in der Primärtherapie ist noch nicht abschließend geklärt. Für Patienten über 60 Jahre ist noch keine kurative Therapie etabliert. Außerdem spielt die Toxizität eine noch größere Rolle. Bei diesen Patienten kann eine MTX-basierte Chemotherapie, z. B. in Kombination mit Ifosfamid oder Temozolomid, eingesetzt werden. Die alleinige Radiotherapie in der Primärbehandlung wird nicht empfohlen. Ihr Stellenwert in Kombination mit einer MTX-basierten Chemotherapie ist ebenfalls nicht etabliert, sie ist jedoch insbesondere bei älteren Patienten mit einer hohen Rate neurotoxischer Spätfolgen verbunden. Im Rezidiv gibt es in der Regel gute Behandlungsmöglichkeiten, deren Auswahl von der Primärtherapie abhängt. In Deutschland gibt es mehrere Studienkonsortien, die wesentliche Arbeiten zur Optimierung der Therapie dieser Tumoren geleistet haben und die sich in enger Diskussion austauschen.

Abstract

Primary CNS lymphomas (PCNSL) are rare brain tumors comprising only 3% of all primary intracranial neoplasms. The median age at diagnosis ranges between 60 and 65 years. PCNSLs often cause a rapidly progressing psychosyndrome. Magnetic resonance imaging (MRI) reveals multiple or single lesions with homogenous contrast enhancement often in the vicinity of the ventricles. The diagnostic method of choice is stereotactic biopsy. In more than 90% of cases a highly malignant B-cell non-Hodgkin’s lymphoma of the diffuse large-cell type is diagnosed. Patients should be included in clinical studies in order to optimize therapy. Patients younger than 60 years old are expected to be treated curatively and should receive polychemotherapy including systemic high-dose methotrexate (MTX). The role of high-dose chemotherapy with autologous stem cell transplantation as primary treatment has not finally been defined. For patients older than 60 years a curative treatment strategy is not established and in addition, toxicity plays a major role. A MTX-based chemotherapy, e.g. in combination with ifosfamide or temozolomide can be administered. Radiotherapy alone as primary treatment is not recommended. Combination radiotherapy with MTX-based chemotherapy is also not established, demonstrating a high rate of neurotoxicity at late follow-up. In the case of recurrence there are a number of possible treatment options depending on the primary therapy. In Germany, several study groups are organized and the aim of these consortia is optimization of PCNSL therapy.

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Authors and Affiliations

  1. Neurologische Klinik am Knappschaftskrankenhaus Bochum-Langendreer, Klinikum der Ruhr-Universität Bochum, In der Schornau 23-25, 44892, Bochum, Deutschland

    U. Schlegel

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

    A. Korfel & E. Thiel

  3. Abt. für Neuroonkologie, Neurologische Universitätsklinik Heidelberg, Heidelberg, Deutschland

    W. Wick

  4. Klinik für Strahlentherapie und Radioonkologie, Universitätsklinikum Leipzig, Leipzig, Deutschland

    R. Kortmann

  5. Institut für Neuropathologie, Universitätsklinik Köln, Köln, Deutschland

    M. Deckert

  6. Neurochirurgische Klinik, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Deutschland

    G. Schackert

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  1. U. Schlegel
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  2. A. Korfel
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  3. E. Thiel
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  4. W. Wick
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  5. R. Kortmann
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  6. M. Deckert
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  7. G. Schackert
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Correspondence to U. Schlegel.

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Schlegel, U., Korfel, A., Thiel, E. et al. Primäre ZNS-Lymphome. Onkologe 15, 211–221 (2009). https://doi.org/10.1007/s00761-009-1573-8

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