Zusammenfassung
Kraniopharyngeome sind seltene dysontogenetische Mittellinienfehlbildungen mit raumforderndem Charakter. Bei häufig langer Anamnesedauer sind Leitsymptome der Erkrankung Kopfschmerzen, Sehstörungen, Wachstumsstörungen und Polyurie/Polydipsie. Der Versuch einer kompletten Resektion unter Wahrung der optischen und hypothalamisch-hypophysären Funktion stellt die Therapie der ersten Wahl bei günstiger Lokalisation des Kraniopharyngeoms im Kindes- und Jugendalter dar. Bei ungünstiger Lokalisation (Hypothalamusbeteiligung) wird eine begrenzte Resektion mit anschließender Strahlentherapie empfohlen. Bei hoher Überlebensrate (93 % Gesamtüberleben) wird die Lebensqualität der Patienten besonders durch tumor- und/oder therapiebedingte hypothalamische Ausfälle beeinträchtigt. Der hypothalamischen Adipositas kommt diesbezüglich eine besondere Bedeutung zu. Die Therapieoptionen zur Behandlung der hypothalamischen Adipositas sind limitiert, sodass die Prävention therapiebedingter (hypothalamischer) Läsionen im Mittelpunkt interdisziplinärer Therapiekonzepte steht. Die Behandlung und Nachsorge der Patienten sollte im Kontext multizentrischer Studien durch erfahrene interdisziplinäre Teams erfolgen.
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Notes
- 1.
Der Abschnitt „Operative Befunde“ wurde von N. Sörensen und J. Krauß verfasst
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Müller, H.L., Sörensen, N., Krauß, J. (2018). Kraniopharyngeome bei Kindern. In: Bächli, H., Lütschg, J., Messing-Jünger, M. (eds) Pädiatrische Neurochirurgie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-48700-6_41
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