Abstract
Paragangliome im Kopf-/Halsbereich treten sporadisch und hereditär auf. Der hereditäre Phänotyp manifestiert sich oftmals zwischen der 2. und 3. Lebensdekade mit unterschiedlicher Penetranz, der sporadische Phänotyp jenseits der 4. Lebensdekade. Der hereditäre Phänotyp „Paragangliomsyndrom Typ 1“ zeigt häufig eine multilokuläre Tumormanifestation und selten Fälle einer Maltransformation. Paragangliome stellen somit eine Systemerkrankung dar. Die Diagnostik erfordert daher ein interdisziplinäres abgestuftes Konzept, das ein genetisches Screening und eine ganzkörperliche internistische Untersuchung einschließlich des Katelcholaminstoffwechsels, optional einer 18F-Dopa-PET-Untersuchung und gezielter Schichtbilduntersuchungsverfahren beinhaltet .
Abstract
Paragangliomas of the head and neck occur sporadically or are hereditary. The hereditary phenotype characteristically occurs between the 2nd and 3rd decade of life; the sporadic phenotype beyond the 4th decade. The hereditary phenotype “paraganglioma syndrome type 1” frequently shows multilocular tumor manifestations and rarely cases of maltransformation. Therefore, paragangliomas should be considered a systemic disease. For diagnosis, an interdisciplinary, step by step procedure is mandatory comprising genetic screening, whole body medical check-up including catecholamine metabolism, and optionally an 18F-Dopa-PET and localised imaging procedures.
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Schipper, J., Boedeker, C.C., Maier, W. et al. Paragangliome im Kopf-/Halsbereich. HNO 52, 569–576 (2004). https://doi.org/10.1007/s00106-003-1007-7
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DOI: https://doi.org/10.1007/s00106-003-1007-7