Kindliche Hirntumoren bei Erwachsenen: Medulloblastom

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Kompendium Internistische Onkologie

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Zusammenfassung

Medulloblastome gehören bei Kindern mit zu den häufigsten Tumoren, bei Erwachsenen sind sie selten. Das Altersmaximum liegt bei 5 Jahren. Nach der aktuellen WHO Klassifikation werden vier histologischen Subgruppen unterschieden. Zudem spielt die molekulare Charakterisierung von Medulloblastomen (SHH-aktiviert p53 wt, SHH-aktiviert p53 mutiert, WNT-aktiviert, Gruppe 3, Gruppe 4) eine diagnostisch und prognostisch wichtige Rolle. Erwachsene mit Medulloblastom präsentieren sich häufig mit einer kurzen Anamnese von Kopfschmerzen, Übelkeit und Erbrechen, Rumpf- oder Extremitätenataxie, zerebellärer Dysarthrie sowie einer Verschlechterung des Allgemeinzustands. Die Diagnostik erfolgt mit MRT der Neuroachse ohne und mit Kontrastmittel. Zum Ausschluss einer mikroskopischen Metastasierung sollte lumbal gewonnener Liquor untersucht werden. Medulloblastome können in seltenen Fällen in Lymphknoten, Lunke, Knochen und Knochenmark metastasieren. In letzter Zeit werden auch bei Erwachsenen die Operation und Strahlentherapie mit einer Chemotherapie kombiniert. Bei Patienten mit SHH-aktivierten Tumoren ist eine zielgerichtete Therapie möglich. Die Nachsorge erfolgt mit MRT des Gehirns, ab M1 auch der Neuroachse, bis 2 Jahre nach Diagnosestellung 3-monatlich, bis Jahr 5 6-monatlich und dann jährlich. Kognitive Aspekte, die Langzeittoxizität der Therapie und soziale und berufliche Rehabilitation stehen im Fokus der Nachsorge.

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Hau, P., Schackert, G., Seidel, C., Wick, W., Weller, M. (2021). Kindliche Hirntumoren bei Erwachsenen: Medulloblastom. In: Schmoll, HJ. (eds) Kompendium Internistische Onkologie . Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-46764-0_381-1

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