Paragangliome – Essentials für den HNO-Arzt

Paragangliome des Kopf-Hals-Bereichs sollten frühzeitig erkannt und an spezialisierten Zentren diagnostiziert und behandelt werden. Dieser Beitrag fokussiert auf die wichtigsten Grundlagen dieses komplexen Krankheitsbilds und beinhaltet Empfehlungen hinsichtlich genetischer Tests, der Diagnostik multizentrischer Tumoren, des multidisziplinären Behandlungsansatzes und der Notwendigkeit einer lebenslangen Überwachung bei der syndromatischen Form.

Abstract

Paragangliomas of the head and neck area should be detected early and diagnosed in their complexity at specialized centers, treated and, in the presence of paraganglioma syndrome, cared for for life. The continuing education article focuses in an abridged form on the most important basics of a complex clinical picture and deals with both sporadic head and neck paragangliomas and hereditary paraganglioma syndromes. It contains concise recommendations regarding genetic testing, the diagnosis of multicentric tumors, the multidisciplinary treatment approach and the need for lifelong monitoring.

Kernaussagen

• Kopf-Hals-Paragangliome sind seltene, aber klinisch bedeutsame Tumoren, die eine sorgfältige diagnostische und therapeutische Herangehensweise erfordern.

• Die neue WHO-Einteilung von 2022 bringt wichtige Änderungen und Klarstellungen, insbesondere in Bezug auf die genetische Klassifikation und die Risikoeinschätzung.

• Die molekulare Klassifikation und moderne bildgebende Verfahren spielen eine zentrale Rolle in der Diagnose und Behandlung.

• Die Unterscheidung zwischen sporadischen Paragangliomen und Paragangliom-Syndromen ist für die weitere Diagnostik und Therapieplanung entscheidend.

• Paragangliom-Syndrome und multizentrische Paragangliome erfordern einen multidisziplinären Ansatz, um optimale Ergebnisse zu erzielen und Prognose sowie Lebensqualität der betroffenen Patienten zu verbessern.

Schlüsselwörter

Paragangliom - Kopf-Hals-Paragangliom - Paragangliomsyndrom - WHO-Einteilung - Screening

Keywords

Paraganglioma - Head and Neck Paraganglioma - Paraganglioma Syndrome - WHO Classification - Screening

Publication History

Article published online:
03 February 2025

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