Endocrine tumors associated with the vagus nerve

Abstract

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 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 aorto-pulmonary window require a thoracic approach. This review particularly emphasizes the embryology, molecular genetics, and modern imaging of these tumors.

AHNS Series: Do you know your guidelines? Optimizing outcomes in reoperative parathyroid surgery: Definitive multidisciplinary joint consensus guidelines of the American Head and Neck Society and the British Association of Endocrine and Thyroid Surgeons

“…Failed parathyroid operations often have identifiable anatomic or technical reasons (ie, a low lying superior gland). Missed adenomas are usually in “normal” positions, although their size over time may promote additional descent into the central neck compartment . A large superior gland will often sink into a paraesophageal location posterior to the recurrent laryngeal nerve.…”

Vagal Paraganglioma: Surgical Removal with Superior Laryngeal Nerve Preservation

“…The vagus nerve descends vertically through the neck within the carotid sheath and within the mediastinum following different pathways on the left and the right sides. cell bodies of fibers that transmit visceral stimuli and regulate “rest and digestive” functions of the parasympathetic system are located in the inferior ganglion of the vagus nerve, and this nerve constitutes the pathway of communication between the central nervous system and the viscera and transmits >80% of sensory input [6].…”

“…However, the expression of mutations in the succinate dehydrogenase subunit D (SDHD) gene is unclear. Familial PGL syndromes commonly occur in young patients [5,6].…”

Vagal Paraganglioma: A Rare Finding in a 31-Year-Old Male

“…Vagal paragangliomas are rare findings that develop from paraganglionic tissue found around the vagus nerve [1]. They are derived from neural crest cells found at or below the inferior ganglion vagus nerve, at the level of the carotid bifurcation, or along the vagus nerve in the mediastinum [2]. The most common paragangliomas of the head and neck include the carotid body tumor, followed by jugulotympanic paragangliomas, and thirdly vagus nerve tumors.…”