Glomus del vago

Abstract

Introduction: Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia. Head and neck paragangliomas are typically associated with blood vessels and neural structures, they arise from the carotid body, jugulotympanic and vagal paraganglia and laryngeal paraganglia. Paragangliomas are benign in almost all cases but symptomatology can be considerable and it is mainly caused by growth towards delicate surrounding structures such as cranial nerves and large vessels. Most paragangliomas are nonfunctional but measurement of urinary vanillylmandelic acid is recommended to avoid problems. Most common presentations of vagal paragangliomas are a painless cervical mass behind the angle of the mandible, dysphonia, dysphagia and vocal paralysis. Imaging studies (computed tomography, magnetic resonance imaging, angiography) are essential in head and neck paragangliomas diagnosis. Vagal paragangliomas have a high degree of vascularity and displace internal carotid artery medially and anteriorly. Most paragangliomas appear to be sporadic, however, some of them are associated with an inherited syndrome. Therefore, genetic testing is recommended for all patients with paragangliomas. Management of head and neck paragangliomas include surgery, radiotherapy and radiosurgical approaches, especially in high-risk patients, incomplete exeresis or recurrent, bilateral or metastatic tumors. A significant number of paragangliomas do not experience any growth, which justifies a wait-and-see policy with annual imaging studies. Methods: We report a 51-year-old male patient with a 6 month history of lip asymmetry and sense of vibration in left periocular area. Neck physical examination was normal. Magnetic resonance imaging (MRI) showed a 27 x 18 x 23 mm mass with well-defined borders in left parapharyngeal-retrostyloid space. T2 weighted image showed a hypointense signal and heterogeneous enhancement after administration of intravenous gadolinium contrast. The mass displaced forward the internal carotid artery and backwards the internal jugular vein, but did not infiltrate nearby structures. These findings were suggestive of vagal paraganglioma. Measurement of urinary vanillylmandelic acid and genetic testing have been requested but the results are not yet available. The patient has been referred to a referral hospital where a wait-and-see approach has been decided. Conclusion: Paragangliomas are benign, slow-growing tumors that can infiltrate nearby structures. Paraganglioma should be considered as a differential diagnosis for painless lateral neck masses. Imaging studies are essential for diagnosis and catecholamines measurment and to look for association with inherited síndromes are mandatory. Treatment options include surgical resection, radiation therapy or wait-and-see policy.