gms | German Medical Science

81. Jahresversammlung der Deutschen Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e. V.

Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e. V.

12.05. - 16.05.2010, Wiesbaden

Paragangliomas of the head and neck: a report on 163 patients (1989–2009)

Meeting Abstract

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  • corresponding author presenting/speaker Konstantinos Papaspyrou - University Medical Center of the Johannes Gutenberg University Mainz, Department of Otorhinolaryngology, Head and Neck Surgery, Mainz, Germany
  • author Torsten Mewes - University Medical Center of the Johannes Gutenberg University Mainz, Department of Otorhinolaryngology, Head and Neck Surgery, Mainz, Germany
  • author Wolf Mann - University Medical Center of the Johannes Gutenberg University Mainz, Department of Otorhinolaryngology, Head and Neck Surgery, Mainz, Germany

German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. 81st Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. Wiesbaden, 12.-16.05.2010. Düsseldorf: German Medical Science GMS Publishing House; 2010. Doc10hno032

DOI: 10.3205/10hno032, URN: urn:nbn:de:0183-10hno0329

Veröffentlicht: 6. Juli 2010

© 2010 Papaspyrou et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

Text

Introduction: Paragangliomas (PGL) of the head and neck are rare, mostly benign tumors. They can arise multifocally and show in 1–3% clinical signs of hormonal activity. 10% to 15% of paragangliomas are caused by mutations in the succinate dehydrogenase (SDH) genes B, C, or D.

Methods: Retrospective study of 163 patients with 206 head and neck PGL, including 59 carotid body, 23 vagal, 83 jugulotympanic, 28 tympanic tumors and 13 PGLs in other locations in the head and neck area, 28 PGLs outside the head and neck area and two papillary thyroid carcinomas. Genetic analysis could be performed on 69 patients.

Results: One patient was found positive for a mutation of von Hippel Lindau (VHL) gene and 30 for succinate dehydrogenase (SDH) gene mutations (19 in SDHD, 7 in SDHC and 4 in SDHB), 16 of the latter carrying a novel mutation. Thirty-three patients (20%) had multiple PGLs and ten (6%) a malignant paraganglioma. Patients with familial mutations had multiple tumors in 61% and malignant tumors in 16%.

Conclusion: Multifocal occurrence, malignancy, hormone production, potential coincidence of thyroid and renal carcinoma and genetic background have to be considered in patients with PGLs of the head and neck.