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Therapie von ZNS-Lymphomen

Therapy of CNS lymphomas

  • Leitthema
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Der Onkologe Aims and scope

Zusammenfassung

Das seltene primäre ZNS-Lymphom (PZNSL) unterscheidet sich von nodalen Lymphomen mit der gleichen Histologie – zumeist handelt es sich um ein diffuses großzelliges B-Zell-Lymphom – durch starke Bindung an das ZNS, sehr aggressiven Verlauf und ungewöhnliche Empfindlichkeit auf Hochdosis-Methotrexat (HDMTX). Die Primärtherapie des PZNSL basiert daher heutzutage auf HDMTX, wobei das optimale Protokoll bisher aufgrund der Seltenheit der Erkrankung nicht etabliert werden konnte. Bei jüngeren Patienten sollte eine Heilung angestrebt und daher ein intensiviertes Chemotherapieprotokoll erwogen werden. Die Ganzhirnbestrahlung verlängert nicht das Gesamtüberleben und sollte daher nicht routinemäßig in der Primärtherapie verwendet werden. Das ebenfalls seltene sekundäre ZNS-Lymphom – definiert als eine ZNS-Beteiligung eines systemischen Lymphoms – hat ebenfalls eine sehr ungünstige Prognose. Die wenigen verfügbaren Daten sprechen für eine Verlängerung des Überlebens durch HDMTX-basierte systemische Chemotherapie und Hochdosischemotherapie, gefolgt von Stammzelltransplantation. Ein wichtiges Therapieziel bei allen Patienten mit einem ZNS-Lymphom ist die Vermeidung einer späten Neurotoxizität.

Abstract

Primary central nervous system lymphoma (PCNSL) differs from nodal lymphoma with similar histological findings, usually diffuse large B-cell lymphoma, by its strong affinity for the central nervous system (CNS), aggressive course and unusual sensitivity to high-dose methotrexate (HDMTX). Thus, primary therapy of PCNSL is currently based on HDMTX but the optimal chemotherapy regimen has not yet been defined due to the rarity of this disease. In younger patients a cure should be the goal and thus intensified chemotherapy protocols should be considered. Whole brain irradiation does not prolong overall survival when used in primary therapy and thus should not be used routinely. Similar to PCNSL the infrequent CNS involvement of a systemic lymphoma, also called secondary CNS lymphoma, has a very poor prognosis. The scarce data suggest a role for HDMTX-based systemic chemotherapy and high-dose chemotherapy followed by stem cell transplantation for outcome improvement. Avoidance of late neurotoxicity is an important goal in the treatment of PCNSL.

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  1. Klinik für Hämatologie und Onkologie, Charité – Universitätsmedizin Berlin, Hindenburgdamm 30, 12200, Berlin, Deutschland

    Agnieszka Korfel

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Korfel, A. Therapie von ZNS-Lymphomen. Onkologe 17, 818–826 (2011). https://doi.org/10.1007/s00761-011-2131-8

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  • DOI: https://doi.org/10.1007/s00761-011-2131-8

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