Zusammenfassung
Hintergrund
Der Befall der Hirnhäute im Rahmen von Karzinomen – Meningeosis carcinomatosa – ist eine in ihrer Häufigkeit unterschätzte Komplikation meist fortgeschrittener Erkrankungen.
Fragestellung
Es werden Inzidenz und Mortalität der Menigeosis carcinomatosa, ihre klinische Präsentation, Diagnostik sowie die aktuellen Therapieempfehlungen dargestellt.
Material und Methode
Für den vorliegenden Beitrag wurden die verfügbare Literatur ausgewertet, eigene Beobachtungen beschrieben sowie Grundlagenarbeiten und Expertenempfehlungen diskutiert.
Ergebnisse
Die hohe Variabilität der klinischen Symptome beruht darauf, dass ganz unterschiedliche Abschnitte des Zentralnervensystems (ZNS) befallen sein können. Viele Patienten sind asymptomatisch, und die Diagnose wird erst bei der Obduktion gestellt. Zu Lebzeiten fußt die Diagnose auf der Kombination aus Magnetresonanztomographie und Liquordiagnostik, wobei beide Methoden immer kombiniert werden sollten. Da das ZNS als anatomisch abgetrennter Raum anzusehen ist – „sanctuary site“ – sind systemische Chemotherapien in Standarddosis oft unwirksam. Therapeutische Ansätze umfassen daher die intrathekale Gabe von Zytostatika, Strahlentherapie und in Einzelfällen chirurgische Maßnahmen.
Schlussfolgerung
Die Prognose der Meningeosis ist meist sehr ungünstig und in der Mehrzahl der Fälle infaust. Nur wenige Patienten überleben langfristig; das mediane Überleben liegt bei weniger als 3 Monaten.
Abstract
Background
The incidence of leptomeningeal involvement from carcinomas, meningeal carcinomatosis, is underestimated and often an event of advanced stages of the disease.
Objective
The incidence and mortality in patients with meningeal carcinomatosis, description of the clinical presentation, diagnostic procedures and current treatment recommendations.
Material and methods
Analysis of the available literature, own observations as well as discussion of review articles and expert recommendations.
Results
The great variability of clinical symptoms is because completely different sections of the central nervous system (CNS) can be affected. In many cases diagnosis is hampered by mild and uncharacteristic or even lack of clinical symptoms. All regions of the leptomeninges can be involved. To establish the diagnosis magnetic resonance scanning of the skull and the spine is as important as the cytological examination of cerebrospinal fluid. As the CNS is a sanctuary site, which is barely reached by systemic chemotherapy, treatment approaches directed at the CNS, such as intrathecal administration of cytotoxic drugs, radiotherapy and in rare cases surgical procedures such as shunt insertion can be necessary.
Conclusion
The prognosis of patients with leptomeningeal disease is very poor and survival is limited to weeks up to few months mostly due to progressive systemic disease. Long-term remission or even cure is possible using combined treatment approaches only in patients with hematological malignancies, such as lymphoma and leukemia.
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M. Bommer: Honorare für Vorträge von Mundipharma. L. Bullinger gibt an, dass kein Interessenkonflikt besteht.
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Bommer, M., Bullinger, L. Meningeosis carcinomatosa. Onkologe 22, 321–328 (2016). https://doi.org/10.1007/s00761-016-0011-y
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DOI: https://doi.org/10.1007/s00761-016-0011-y