Primäre Lymphome des zentralen Nervensystems

Primäre ZNS-Lymphome sind Non-Hodgkin Lymphome des Gehirnparenchyms, der Meningen, und/oder des Rückenmarks mit fakultativer Beteiligung der Augen. Durch die Verfügbarkeit effektiver Chemotherapieprotokolle hat sich die Prognose dieser Tumoren insbesondere für jüngere Patienten in den letzten Jahrzehnten deutlich verbessert.

Fazit

Take Home Message

Primäre ZNS-Lymphome (PZNSL) sind seltene, bösartige, primäre Hirntumoren.

Histologisch handelt es sich ganz überwiegend um diffus großzellige Non-Hodgkin B-Zell-Lymphome.

Fazit

Take Home Message

Eine alleinige Chemotherapie ist eine wirksame Therapie bei PZNSL. Die Chemotherapie sollte aus einer Induktionstherapie basierend auf HDMTX und einer anschließend intensivierten Konsolidierungstherapie aufgebaut sein.

Fazit

Take Home Message

Die Ganzhirnbestrahlung ist aufgrund erheblicher neurokognitiver Spätfolgen in Deutschland nicht mehr Bestandteil der Erstlinientherapie bei PZNSL.

Fazit

Take Home Message

Der Versuch einer operativen Resektion ist bei PZNSL nicht indiziert.

Fazit

Take Home Message

Die Prognose älterer Patienten mit PZNSL ist deutlich ungünstiger als die Prognose jüngerer Patienten.

Fazit

Take Home Message

Eine etablierte Standardtherapie für die Behandlung von PZNSL-Rezidiven existiert nicht.

Kernaussagen

• Primäre ZNS-Lymphome (PZNSL) sind seltene maligne Tumoren des zentralen Nervensystems und überwiegend den diffus großzelligen B-Zell-Non-Hodgkin-Lymphomen zuzuordnen

• Die operative Entfernung des Tumors spielt keine Rolle.

• Die Strahlentherapie sollte in der Primärtherapie und im Rezidiv aufgrund neurotoxischer Spätfolgen wann immer möglich vermieden werden.

• Eine alleinige Chemotherapie basierend auf Hochdosis-Methotrexat (HDMTX) führt insbesondere bei jüngeren Patienten häufig zu Langzeitremissionen.

• Die Chemotherapie sollte aus einem Induktions- und einem Konsolidierungsteil bestehen.

• Im Rezidiv besteht keine etablierte Standardtherapie, jedoch stellen hier neue gezielte Therapieansätze, die in klinischen Studien geprüft werden, interessante Möglichkeiten dar.

Publication History

Article published online:
26 February 2021

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