Evaluation of Head and Neck Paragangliomas by Computed Tomography in Patients with Pheochromocytoma-Paraganglioma Syndromes.

Autor:	Michałowska I; Department of Radiology, Institute of Cardiology, Warsaw, Poland., Lewczuk A; Department of Endocrinology and Internal Medicine, Medical University of Gdańsk, Gdańsk, Poland., Ćwikła J; Department of Nuclear Medicine, Faculty of Medical Science, University of Varmia and Masuria, Olsztyn, Poland., Prejbisz A; Department of Hypertension, Institute of Cardiology, Warsaw, Poland., Swoboda-Rydz U; Department of Foreign Languages, Medical University of Warsaw, Warsaw, Poland., Furmanek MI; Department of Radiology and Diagnostic Imaging, Medical Centre for Postgraduate Education, Warsaw, Poland., Szperl M; Department of Laboratory of Molecular Biology, Institute of Cardiology, Warsaw, Poland., Januszewicz A; Department of Hypertension, Institute of Cardiology, Warsaw, Poland., Pęczkowska M; Department of Hypertension, Institute of Cardiology, Warsaw, Poland.
Jazyk:	angličtina
Zdroj:	Polish journal of radiology [Pol J Radiol] 2016 Oct 31; Vol. 81, pp. 510-518. Date of Electronic Publication: 2016 Oct 31 (Print Publication: 2016).
DOI:	10.12659/PJR.897490
Abstrakt:	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. Material/methods: A total of 72 patients with SDHx mutations underwent computed tomography examinations of the head and neck. HNP were present in 44 (61.1%) out of 72 patients (31 SDHD , 11 SDHB , 2 SDHC ); 113 HNP were found; the most common were carotid paragangliomas (59) and vagal paragangliomas (27). Results: The HNP were statistically more frequent in carriers of SDHD mutations compared to carriers of SDHB mutations (72.1% vs. 43.5%, p=0.033). Multiple tumors more often occurred in patients with SDHD mutations 26/31 (83.9%) than in patients with SDHB mutations 6/11 (54.5%) p=0.05. There was a significant difference in the prevalence of carotid paragangliomas between patients with SDHB and SDHD mutations (7/11 [63.6%] vs. 30/31 [96.8%], respectively, p=0.004). Patients with SDHD mutations more often had carotid paragangliomas located on the left side than on the right side, as compared to SDHB mutations 25/31 (80.6%) vs. 4/11 (36.4%), p=0.006. Conclusions: SDHx mutations predispose to multifocal and bilateral HNP. Carotid and vagal paragangliomas occurred most often. Patients with SDHD mutations are characterized by higher frequency of HNP than patients with SDHB mutations, which is mainly driven by higher frequency of carotid body tumors in patients with SDHD mutations. No difference in the frequency of head and neck paragangliomas in other locations was found. Competing Interests: The authors declare that they have no conflict of interest.
Databáze:	MEDLINE
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