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Adipositas - Ursachen, Folgeerkrankungen, Therapie 2015; 09(01): 34-39
DOI: 10.1055/s-0037-1618892
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Schattauer GmbH

Hypothalamische Adipositas bei Kraniopharyngeom im Kindes- und Jugendalter

Hypothalamic obesity in childhood craniopharyngioma
H. L. Müller
1   Klinik für Allgemeine Kinderheilkunde, Hämatologie / Onkologie, Zentrum für Kinder- und Jugendmedizin, Klinikum Oldenburg, Medizinischer Campus Universität Oldenburg, Oldenburg
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Publication History

Publication Date:
21 December 2017 (online)

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Zusammenfassung

Kraniopharyngeome sind seltene embryonale Mittellinienfehlbildungen mit raumforderndem Charakter. Bei häufig langer Anamnesedauer sind Leitsymptome der Erkrankung: Sehstörungen, Kopfschmerzen, Wachstumsstörungen und Polyurie/Polydypsie (Diabetes insipidus neurohormonalis). Der Versuch einer kompletten Resektion unter Wahrung der optischen und hypothalamisch-hypophysären Funktion stellt die Therapie der ersten Wahl bei günstiger Lokalisation des Fehlbildungstumors dar. Bei ungünstiger Lokalisation (Hypothalamusbeteiligung) wird eine begrenzte Resektion mit anschließender Strahlentherapie empfohlen. Bei hoher Überlebensrate (93 %) wird die Lebensqualität der Patienten insbesondere durch eine hypothalamische Adipositas beeinträchtigt. Die Therapieoptionen zur Behandlung der hypothalamischen Adipositas sind eingeschränkt, so dass der Prävention therapiebedingter hypothalamischer Läsionen eine besondere Bedeutung zukommt.

Summary

Childhood craniopharyngiomas are sellar embryogenic malformations of low-grade histological malignancy and low incidence. Despite high survival rates (92 %), the quality of survival is frequently impaired due to sequelae caused by hypothalamic obesity. Recent reports have indicated that the degree of obesity of affected patients is positively correlated with the degree and extent of hypothalamic damage. Therefore, craniopharyngioma can be considered as a paradigmatic disease, reflecting challenges in diagnostic and treatment of hypothalamic obesity. Despite the availability of promising therapeutic approaches, it must be emphasized that there is currently no pharmacological therapy for hypothalamic obesity in craniopharyngioma that has been shown to be effective in controlled studies. Multidisciplinary strategies aiming at preservation of hypothalamic integrity are mandatory for the prevention of hypothalamic obesity.

Schlüsselwörter

Kraniopharyngeom - Adipositas - Hypothalamus - bariatrische Operationen - zentrale Stimulanzien

Keywords

Craniopharyngioma - obesity - hypothalmus - bariatric surgery - central stimulating agents
 

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