Head and neck paragangliomas: Report of 175 patients (1989-2010)

• Published in: Head & neck Vol. 34; no. 5; pp. 632 - 637
• Main Authors: Papaspyrou, Konstantinos, Mewes, Torsten, Rossmann, Heidi, Fottner, Christian, Schneider-Raetzke, Brigitte, Bartsch, Oliver, Schreckenberger, Mathias, Lackner, Karl J., Amedee, Ronald G., Mann, Wolf J.
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01-05-2012; Wiley
• Subjects: Biological and medical sciences; Carcinoma, Papillary - pathology; Female; genetic analysis; head and neck; Head and Neck Neoplasms - genetics; Head and Neck Neoplasms - pathology; Heterozygote; Humans; Male; malignancy; Medical sciences; Mutation; Neoplasms, Multiple Primary - pathology; Neurology; Otorhinolaryngology. Stomatology; Paraganglioma - genetics; Paraganglioma - pathology; paragangliomas; Retrospective Studies; succinate dehydrogenase; Succinate Dehydrogenase - genetics; Tumors of the nervous system. Phacomatoses; Von Hippel-Lindau Tumor Suppressor Protein - genetics; Human; Nervous system diseases; Enzyme; Stomatology; Paraganglioma; Malignancy; ENT; Malignant tumor; Dehydrogenase; succinate dehydrogenase; Case study; genetic analysis; paragangliomas; Diseases of the autonomic nervous system; Head and neck; Genetics; Oxidoreductases; Cancer
• ISSN: 1043-3074; 1097-0347
• DOI: 10.1002/hed.21790

Background
Attention of the otorhinolaryngologist needs to be drawn to the versatile aspects of head and neck paragangliomas (PGLs).
Methods
This study is a retrospective, nonrandomized clinical study of all 175 individuals with PGLs treated in our department between 1989 and 2010. A genetic analysis was performed on 86 patients.
Results
The 175 patients presented 224 head and neck PGLs as well as 2 thyroid papillary carcinomas. Genetic analysis resulted in 1 patient positive for a von Hippel‐Lindau (VHL) gene mutation and 34 for succinate dehydrogenase (SDH) gene mutations (22 SDHD, 7 SDHC, and 5 SDHB), 12 of the latter carrying a novel mutation. Thirty‐three patients (18.9%) had multiple PGLs and 11 patients (6.3%) had a malignant paraganglioma. SDH‐mutation carriers had multiple tumors in 64.7% and malignant paragangliomas in 20.6%.
Conclusions
Multifocal occurrence, potential malignancy, genetic aspects, possible coincidence of thyroid carcinoma, and hormone production have to be considered in patients with head and neck PGLs. © 2011 Wiley Periodicals, Inc. Head Neck, 2012