Papaspyrou, Konstantinos; Mewes, Torsten; Rossmann, Heidi; Fottner, Christian; Schneider-Raetzke, Brigitte; Bartsch, Oliver; Schreckenberger, Mathias; Lackner, Karl J.; Amedee, Ronald G.; Mann, Wolf J.
Abstract
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. (C) 2011 Wiley Periodicals, Inc. Head Neck 34: 632-637, 2012
