title: Evaluation of Head and Neck Paragangliomas by Computed Tomography in Patients with Pheochromocytoma-Paraganglioma Syndromes.
abstract: BACKGROUND
  Hereditary head and neck paragangliomas (HNP) are very often associated with pheochromocytoma-paraganglioma syndromes, which are caused by mutations in genes encoding subunits of succinate dehydrogenase (SDHx) complex. The aim of this study was to determine the frequency and location of HNP among SDHx carriers.
  
  
  METHODS
  A total of 72 patients withSDHxmutations underwent computed tomography examinations of the head and neck. HNP were present in 44 (61.1%) out of 72 patients (31SDHD, 11SDHB, 2SDHC); 113 HNP were found; the most common were carotid paragangliomas (59) and vagal paragangliomas (27).
  
  
  RESULTS
  The HNP were statistically more frequent in carriers ofSDHDmutations compared to carriers ofSDHBmutations (72.1%vs.43.5%, p=0.033). Multiple tumors more often occurred in patients withSDHDmutations 26/31 (83.9%) than in patients withSDHBmutations 6/11 (54.5%) p=0.05. There was a significant difference in the prevalence of carotid paragangliomas between patients withSDHBandSDHDmutations (7/11 [63.6%]vs.30/31 [96.8%], respectively, p=0.004). Patients withSDHDmutations more often had carotid paragangliomas located on the left side than on the right side, as compared toSDHBmutations 25/31 (80.6%)vs.4/11 (36.4%), p=0.006.
  
  
  CONCLUSIONS
  SDHxmutations predispose to multifocal and bilateral HNP. Carotid and vagal paragangliomas occurred most often. Patients withSDHDmutations are characterized by higher frequency of HNP than patients withSDHBmutations, which is mainly driven by higher frequency of carotid body tumors in patients withSDHDmutations. No difference in the frequency of head and neck paragangliomas in other locations was found.
authors.affiliation: Department of Radiology, Institute of Cardiology, Warsaw, Poland.
authors.forename: Ilona
authors.initials: I
authors.lastname: Michałowska
authors.affiliation: Department of Endocrinology and Internal Medicine, Medical University of Gdańsk, Gdańsk, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Lewczuk
authors.affiliation: Department of Nuclear Medicine, Faculty of Medical Science, University of Varmia and Masuria, Olsztyn, Poland.
authors.forename: Jarosław
authors.initials: J
authors.lastname: Ćwikła
authors.affiliation: Department of Hypertension, Institute of Cardiology, Warsaw, Poland.
authors.forename: Aleksander
authors.initials: A
authors.lastname: Prejbisz
authors.affiliation: Department of Foreign Languages, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Urszula
authors.initials: U
authors.lastname: Swoboda-Rydz
authors.affiliation: Department of Radiology and Diagnostic Imaging, Medical Centre for Postgraduate Education, Warsaw, Poland.
authors.forename: Mariusz I
authors.initials: MI
authors.lastname: Furmanek
authors.affiliation: Department of Laboratory of Molecular Biology, Institute of Cardiology, Warsaw, Poland.
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Szperl
authors.affiliation: Department of Hypertension, Institute of Cardiology, Warsaw, Poland.
authors.forename: Andrzej
authors.initials: A
authors.lastname: Januszewicz
authors.affiliation: Department of Hypertension, Institute of Cardiology, Warsaw, Poland.
authors.forename: Mariola
authors.initials: M
authors.lastname: Pęczkowska
keywords: Carotid Body Tumor; Head and Neck Neoplasms; Paraganglioma, Extra-Adrenal; Succinate Dehydrogenase