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Von-Hippel-Lindau-Syndrom

Von Hippel-Lindau syndrome

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Zusammenfassung

Das Von-Hippel-Lindau-Syndrom ist eine autosomal dominant vererbte Phakomatose mit der Prädisposition ZNS und Retina sowie variabler Ausprägung mit Hämangioblastomen von Kleinhirn, Medulla oblongata, Rückenmark, Nierenkarzinomen, Phäochromozytomen, Pankreaszysten und Inselzelltumoren sowie Tumoren des Endolymphsacks des Innenohrs [1]. Etwa ab dem 30. Lebensjahr treten klinische Symptome auf. Eine MRT-Untersuchung der Neuroaxis ist bei allen Patienten mit Verdacht auf eine Von-Hippel-Lindau-Erkrankung indiziert.

Abstract

Von Hippel-Lindau syndrome is an autosomal dominant inherited phacomatosis with a predisposition for the central nervous system and retina. There is variable expression with hemangioblastomas in the brain, medulla oblongata, spinal chord, renal carcinoma, pheochromocytoma, pancreatic cysts and islet cell tumors as well as tumors of the endolymphatic sac of the inner ear. Clinical symptoms occur first after an age of approximately 30 years. Magnetic resonance imaging (MRI) of the neuroaxis is indicated in all patients with a suspicion of von Hippel-Lindau syndrome.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. W. Reith und H. Körner geben an, dass kein Interessenkonflikt besteht.

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Authors and Affiliations

  1. Klinik für Diagnostische und Interventionelle Neuroradiologie, Universitätsklinikum des Saarlandes, 66424, Homburg/Saar, Deutschland

    W. Reith & H. Körner

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  1. W. Reith
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  2. H. Körner
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Correspondence to W. Reith.

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Reith, W., Körner, H. Von-Hippel-Lindau-Syndrom. Radiologe 53, 1104–1106 (2013). https://doi.org/10.1007/s00117-013-2606-2

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  • DOI: https://doi.org/10.1007/s00117-013-2606-2

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