Glomus vagale: Paraganglioma of the vagus nerve

Urquhart, Andrew C.; Johnson, Jonas T.; Myers, Eugene N.; Schechter, Gary L.

doi:10.1288/00005537-199404000-00008

Cited by 78 publications

(11 citation statements)

References 30 publications

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“…At times, this can be heard by placing the stethoscope over the ear. Less than 50% of VPs present with deficits of cranial nerves, which manifest as hoarseness (X), dysphagia (IX), shoulder drop (XI), nasal reflux of fluids, aspiration and hemiatrophy of the tongue (XII) (10),(11). Intracranial extension, which is the main cause of death, occurs in 22% of the cases (4).…”

Section: Signs and Symptomsmentioning

confidence: 99%

Head and neck paragangliomas: clinical and molecular genetic classification

Offergeld¹,

Brase²,

Yaremchuk³

et al. 2012

Clinics

159

139

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Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen and thorax, head and neck paragangliomas seldom release catecholamines and are hence rarely vasoactive. Petrous bone, jugular, and tympanic head and neck paragangliomas may cause hearing loss. The internationally accepted clinical classifications for carotid body tumors are based on the Shamblin Class I–III stages, which correspond to postoperative permanent side effects. For petrous-bone paragangliomas in the head and neck, the Fisch classification is used. Regarding the molecular genetics, head and neck paragangliomas have been associated with nine susceptibility genes: NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2 (SDH5), and TMEM127. Hereditary HNPs are mostly caused by mutations of the SDHD gene, but SDHB and SDHC mutations are not uncommon in such patients. Head and neck paragangliomas are rarely associated with mutations of VHL, RET, or NF1. The research on SDHA, SDHAF2 and TMEM127 is ongoing. Multiple head and neck paragangliomas are common in patients with SDHD mutations, while malignant head and neck paraganglioma is mostly seen in patients with SDHB mutations. The treatment of choice is surgical resection. Good postoperative results can be expected in carotid body tumors of Shamblin Class I and II, whereas operations on other carotid body tumors and other head and neck paragangliomas frequently result in deficits of the cranial nerves adjacent to the tumors. Slow growth and the tendency of hereditary head and neck paragangliomas to be multifocal may justify less aggressive treatment strategies.

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Section: Signs and Symptomsmentioning

confidence: 99%

Head and neck paragangliomas: clinical and molecular genetic classification

Offergeld¹,

Brase²,

Yaremchuk³

et al. 2012

Clinics

159

139

View full text Add to dashboard Cite

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“…The probability of surgical cure in VPGLs is also very high: GTR is possible in 92.3-100% of the cases with a low mortality rate (0-2.7%) (Urquhart et al 1994, Netterville et al 1998, Kollert et al 2006. In a large literature review on VPGLs and JTPGLs published in 2012, Suarez et al (2013a) described an average mortality rate of 1.3% with a GTR rate of 93.3%.…”

Section: Vagus Paragangliamentioning

confidence: 99%

“…An immediate postoperative CN palsy rate can be as high as 96 and 100% respectively for these tumours (Neskey et al 2011). The most affected component is the vagus nerve itself: in most series, a postoperative vagal deficit is almost universal (92-100%), by either paresis or necessary sacrifice during surgery (Urquhart et al 1994, Netterville et al 1998 (Suarez et al 2013a). The majority of patients need complex rehabilitation management regarding speech, swallowing and facial nerve deficits, but during follow-up, these deficits often recover partially (Netterville et al 1998).…”

Section: Vagus Paragangliamentioning

confidence: 99%

The management of head-and-neck paragangliomas

Căpățînă

Ntali

Karavitaki

et al. 2013

Endocrine-Related Cancer

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Paragangliomas (PGLs) are tumours originating from neural crest-derived cells situated in the region of the autonomic nervous system ganglia. Head-and-neck PGLs (HNPGLs) originate from the sympathetic and parasympathetic paraganglia, most frequently from the carotid bodies and jugular, tympanic and vagal paraganglia, and are usually non-catecholamine secreting. Familial PGLs are considered to be rare, but recently genetic syndromes including multiple PGLs and/or phaeochromocytomas have been more thoroughly characterised. Nowadays, genetic screening for the genes frequently implicated in both familial and sporadic cases is routinely being recommended. HNPGLs are mostly benign, generally slow-growing tumours. Continuous growth leads to the involvement of adjacent neurovascular structures with increased morbidity rates and treatment-related complications. Optimal management mostly depends on tumour location, local involvement of neurovascular structures, estimated malignancy risk, patient age and general health. Surgery is the only treatment option offering the chance of cure but with significant morbidity rates, so a more conservative approach is usually considered, especially in the more difficult cases. Radiotherapy (fractionated or stereotactic radiosurgery) leads to tumour growth arrest and symptomatic improvement in the short term in many cases, but the longterm consequences are unclear. Early detection is essential in order to increase the chance of cure with a lower morbidity rate. The constant improvement in diagnostic imaging, surgical and radiation techniques has led to a safer management of these tumours, but there are still many therapeutic challenges, and no treatment algorithm has been agreed upon until now. The management of HNPGLs requires a multidisciplinary effort addressing the genetic, surgical, radiotherapeutic, oncological, neurological and endocrinological implications. Further progress in the understanding of their pathogenesis will lead to more effective screening and earlier diagnosis, both critical to successful treatment.

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“…The progression of symptoms is helpful in differentiating vagal paraganglioma from other head neck paraganglioma. 15 Our patient had symptoms of dysphagia, hoarseness and atrophy of left half of the tongue.…”

Section: Discussionmentioning

confidence: 70%

Synchronous malignant vagal paraganglioma with contralateral carotid body paraganglioma treated by radiation therapy

et al. 2010

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Paragangliomas are rare tumors and very few cases of malignant vagal paraganglioma with synchronous carotid body paraganglioma have been reported. We report a case of a 20-year old male who presented with slow growing bilateral neck masses of eight years duration. He had symptoms of dysphagia to solids, occasional mouth breathing and hoarseness of voice. Fine needle aspiration cytology (FNAC) performed where he lived showed a sinus histiocytosis and he was administered anti-tubercular treatment for six months without any improvement in his symptoms. His physical examination revealed pulsatile, soft to firm, non-tender swellings over the anterolateral neck confined to the upper-mid jugulo-diagastric region on both sides. Direct laryngoscopy examination revealed a bulge on the posterior pharyngeal wall and another over the right lateral pharyngeal wall. Magnetic resonance imaging (MRI), 99mTc-labeled octreotide scan and angiography diagnosed the swellings as carotid body paraganglioma, stage III on the right side with left-sided vagal malignant paraganglioma. Surgery was ruled out as a high morbidity with additional risk to life was expected due to the highly vascular nature of the tumor. The patient was treated with radiation therapy by image guided radiation to a dose of 5040cGy in 28 fractions. At a follow-up at 16 months, the tumors have regressed bilaterally and the patient can take solids with ease.

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Glomus vagale: Paraganglioma of the vagus nerve

Cited by 78 publications

References 30 publications

Head and neck paragangliomas: clinical and molecular genetic classification

Head and neck paragangliomas: clinical and molecular genetic classification

The management of head-and-neck paragangliomas

Synchronous malignant vagal paraganglioma with contralateral carotid body paraganglioma treated by radiation therapy

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