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Endocr Relat Cancer. 2016 Sep;23(9):R371-9. doi: 10.1530/ERC-16-0241. Epub 2016 Jul 12.

Endocrine tumors associated with the vagus nerve.

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Department of RadiologyConception Hospital, Aix-Marseille University, Marseille, France.
Endocrine Oncology BranchNational Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
Department of Endocrine SurgeryConception Hospital, Aix-Marseille University, Marseille, France.
Section on Medical NeuroendocrinologyEunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland, USA.
Department of Nuclear MedicineLa Timone University Hospital, CERIMED, Aix-Marseille University, Marseille, France


The vagus nerve (cranial nerve X) is the main nerve of the parasympathetic division of the autonomic nervous system. Vagal paragangliomas (VPGLs) are a prime example of an endocrine tumor associated with the vagus nerve. This rare, neural crest tumor constitutes the second most common site of hereditary head and neck paragangliomas (HNPGLs), most often in relation to mutations in the succinate dehydrogenase complex subunit D (SDHD) gene. The treatment paradigm for VPGL has progressively shifted from surgery to abstention or therapeutic radiation with curative-like outcomes. Parathyroid tissue and parathyroid adenoma can also be found in close association with the vagus nerve in intra or paravagal situations. Vagal parathyroid adenoma can be identified with preoperative imaging or suspected intraoperatively by experienced surgeons. Vagal parathyroid adenomas located in the neck or superior mediastinum can be removed via initial cervicotomy, while those located in the aortopulmonary window require a thoracic approach. This review particularly emphasizes the embryology, molecular genetics, and modern imaging of these tumors.


diagnostic imaging; hyperparathyroidism; paragangliomas; vagus nerve

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