BACKGROUND: A glomus vagale tumor is an infrequent paraganglioma primarily characterized by auditory symptoms, cranial nerve involvement, or autonomic symptoms. However, visual involvement is not commonly observed, and to date, no cases have been reported in the literature.
METHODS: The case involves a 62-year-old female patient with a history of right carotid body tumor resection. She presented to the emergency department with a sudden decrease in visual acuity and bitemporal hemianopsia, accompanied by a left parietal headache. Initial brain magnetic resonance imaging (MRI) revealed a pituitary macroadenoma, which was completely resected. However, postoperatively, the patient developed left amaurosis. Subsequent brain MRI showed the presence of hemostatic material mixed with blood in the sellar region, causing displacement of the optic chiasm. A repeat intervention was performed, identifying bleeding from both cavernous sinuses. Head and neck angiography demonstrated a right glomus vagale tumor with abundant blood drainage into the right cavernous sinus. Embolization of the glomus vagale tumor was performed, resulting in no further bleeding and improvement of symptoms.
CONCLUSIONS: The aim of this case report is to describe a rare occurrence of bilateral visual disturbances caused by bleeding in both cavernous sinuses due to venous hypertension caused by a right glomus vagale tumor.

Vagus nerve paragangliomas are rare tumors, comprising 0.03% of head and neck neoplasms. These tumors are usually located cephalad to the hyoid bone, and there is only one previously reported case that arose from the lower third of the neck.
We describe the second reported case of a lower neck vagus nerve paraganglioma that was managed with a limited sternotomy for access and surgical removal.
A 66-year-old male presented with a long-standing lesion of the cervicothoracic junction. CT, MRI, and Ga-68 DOTATATE PET/CT showed an avidly enhancing 5.2 × 4.2 × 11.5 cm mass extending from C6 to approximately T4 level. FNA confirmed the diagnosis. The patient underwent catheter angiography and embolization via direct puncture technique followed by excision of the mass via a combined transcervical and limited sternotomy approach.
We describe an unusual case of vagal paraganglioma at the cervicothoracic junction with retrosternal extension requiring a sternotomy for surgical excision.

UNASSIGNED: Vagal paragangliomas of neck are rare tumours of neural crest origin usually arising in elderly age with female predominance. They have a vague clinical presentation therefore difficult to diagnose preoperatively. We hope that this case report and literature review would add to the existing literature and help devise a comprehensive diagnostic and therapeutic plan for vagal paragangliomas.
METHODS: We report a case of vagal paraganglioma occurring in a 13-year-old male which is an extremely rare presentation in this age group. The patient presented with a large solitary painless progressively growing mass in posterior triangle of neck. External jugular vein was stretched and trachea was deviated medially. The mass was arising via a twig from vagus nerve and was surgically excised. Diagnosis was established post-operatively through histopathological analysis.
UNASSIGNED: Vagal paraganglioma is a rare occurrence in male teenagers and may mimic schwannoma, neuroma, jugular meningioma, or other gangliomas. Surgical excision is mainstay of treatment but resultant vagal complications and neurological consequences are usually unavoidable. Nonetheless, the prognosis may be easily improved with sound surgical judgement, skill, and routine follow-up.
CONCLUSIONS: Vagal paraganglioma usually presents as a swelling in neck and cannot be diagnosed on simple clinical examination. CT scan and MRI are imaging modalities of choice and can be coupled with angiography to increase diagnostic accuracy. Although both radiation therapy and surgical excision have both been found to be successful treatment options, it is still unclear which is more beneficial.

Vagal paraganglioma is a rare finding that develops from paraganglionic tissue found around the vagus nerve; it has a prevalence of 0.012% of all tumors. It is the third most common paraganglioma of the head and neck but still accounts for less than 5% of these tumors, and it has a well-established female prevalence. It is a difficult tumor to identify early based on its symptoms alone and only a thorough investigation can help solidify its diagnosis. In this report, we discuss a presentation of this phenomenon that is not only unique in its manifestation but also a very difficult diagnosis due to its deceptive presentation and multiple extensions. These masses need a good surgical regime to be removed properly and postoperative complications are very frequent in most of these cases.

Glomus vagale tumor is a paraganglioma of the vagus nerve. It is a rare type of benign tumor that occupies the head and neck and skull base regions. Patients often present with lower cranial nerve dysfunctions such as difficulty swallowing, tongue weakness, and hoarseness. Surgical treatment can be complex and difficult due to its high vascularity, frequent involvement of lower cranial nerves, and surrounding critical vascular structures. In this operative video, we demonstrate an extended anterolateral infralabyrinthine transjugular approach for microsurgical resection of a giant glomus vagale tumor in a 53-year-old male who presented with an enlarging neck mass, difficulty swallowing, right tongue weakness, and hoarseness. Imaging revealed a giant glomus vagale tumor in the right parapharyngeal space extending into the jugular foramen with occlusion of the internal jugular vein. After preoperative embolization, the patient underwent a near-total resection of the tumor with a small microscopic residual at the pars nervosa. In summary, the extended anterolateral infralabyrinthine transjugular approach is a useful strategy for removal of giant glomus vagale tumors extending into the skull base. The surgical technique and nuances are described in a step-by-step fashion in this illustrative operative video. The link to the video can be found at: https://youtu.be/L0EosQK95LE .

Radiation-induced sarcoma is a known but rare complication of radiation treatment for skull base paraganglioma. We present the cases of a female patient with multiple paraganglioma syndrome treated with external beam radiation treatment who presented 4 years later with a malignant peripheral nerve sheath tumor of the vagus nerve.

Head and neck paragangliomas (HNPs) are rare, usually benign hyper vascularized neuroendocrine tumors that traditionally have been treated by surgery, with or without endovascular embolization, or, more recently stereotactic radiosurgery (SRS). The aim of our study is to determine the clinical and radiographic effectiveness of SRS for treatment of HNPs. A systematic search of electronic databases was performed, and 37 articles reporting 11,174 patients (1144 tumors) with glomus jugulare (GJT: 993, 86.9%), glomus tympanicum (GTT: 94, 8.2%), carotid body tumors (CBTs: 28, 2.4%), and glomus vagale (GVT: 16, 1.4%) treated with SRS definitively or adjuvantly were included. The local control (LC) was estimated from the pooled analysis of the series, and its association with SRS technique as well as demographic and clinical factors was analyzed. The median age was 56 years (44-69 years). With a median clinical and radiological follow-up of 44 months (9-161 months), LC was 94.2%. Majority of the patients (61.0%) underwent Gamma Knife Radiosurgery (GKS), but there was no statistically significant difference in LC depending upon the SRS technique (p = 0.9). Spearmen\'s correlation showed that LC was strongly and negatively correlated with multiple parameters, which included female gender (r = - 0.4, p = 0.001), right-sided tumor (r = - 0.3, p = 0.03), primary SRS (r = - 0.5, p ≤ 0.001), and initial clinical presentation of hearing loss (r = - 0.4, p = 0.001). To achieve a LC ≥ 90%, a median marginal dose (Gy) of 15 (range, 12-30 Gy) was required. The results corroborate that SRS in HNPs is associated with good clinical and radiological outcome.

OBJECTIVE: To provide the first description of hypofractionated stereotactic radiosurgery (SRS) and evaluate tumor control and safety for vagal paragangliomas (VPs), which begin at the skull base but often have significant extracranial extension.
METHODS: Retrospective chart review.
METHODS: Tertiary-referral neurotology and neurosurgery practice.
METHODS: Five VPs in 4 patients (all male, ages 15-56 years) underwent SRS between 2010 and 2018. Outcome measures included tumor dimensions on serial imaging, cranial nerve function, and radiation side effects.
RESULTS: CyberKnife hypofractionated SRS was performed. The prescription dose was 24 or 27 Gy (maximum dose 33.4 Gy; range, 29.3-35.5 Gy) delivered in 3 equal fractions. The mean isodose line was 79% (range, 76%-82%). Four VPs were treated primarily, and 1 tumor underwent SRS to treat regrowth 2 years after microsurgical subtotal resection via the modified infratemporal fossa approach. The treatment volume ranged from 8.81 to 86.3 cm3 (mean, 35.7 cm3). All demonstrated stable size (n = 3) or regression (n = 2) at last follow-up, 63 to 85 months after SRS (mean, 76 months). One patient had stable premorbid vocal fold paralysis from a prior ipsilateral glomus jugulare tumor resection. All others demonstrated normal vagal function following SRS. Treatment-related side effects, including dysgeusia (n = 1), mucositis (n = 1), and neck soft-tissue edema (n = 2), were self-limited.
CONCLUSIONS: Hypofractionated SRS appears to be both safe and effective for treating VPs, including large-volume and predominantly extracranial tumors, while preserving vagal function. SRS should be considered as a cranial nerve preservation option, especially in settings of contralateral lower cranial nerve deficits or in those with multiple paragangliomas risking both vagal nerves.

Introducing the diagnostic protocol and therapy of a rare disease reviewing the worldwide literature named as vagal paraganglioma is our main target. This type is specific for women of middle age, and it is appearing as a unilateral neck mass, with or without Fontaine\'s sign. Giving a heavy aspect on the ear, nose and throat examination, the most important diagnostic tool is CT-angiography. Carefully looking at the size and relationship with the narrowing arteries, veins, nerves, muscles and bony structures (skull base, cervical spine), in most of the cases we choose the surgical procedure. Orv Hetil. 2019; 160(34): 1358-1362.
Absztrakt: Esetbemutatásunkban a világviszonylatban is ritkának számító glomus vagale tumorok kivizsgálásáról, terápiájáról adunk összefogó képet. A jellemzően középkorú nőket érintő betegség unilaterálisan elhelyezkedő nyaki tumoros elváltozás képében jelenik meg, melyre nem minden esetben jellemző a felette érezhető pulzáció (Fontaine-jel), a glomus caroticum tumorokkal szemben. Nagy hangsúlyt fektetve a fül-orr-gégészeti fizikális vizsgálatra, a betegség pontos diagnózisát a CT-angiográfia adja meg. Figyelembe véve a kiterjedést, a szomszédos erekhez, idegekhez, izmokhoz, illetve csontszerkezetű képletekhez (koponyaalap, nyaki gerinc) való viszonyt, az esetek nagy részében sebészi beavatkozás mellett döntünk. A bemutatott beteg kezelése 2017 szeptemberében történt Osztályunkon. Orv Hetil. 2019; 160(34): 1358–1362.

Vascular neoplasms of the head and neck present with a wide spectrum of signs and symptoms. Diagnosis requires a high index of suspicion and is usually made after tumors are large enough to be visually apparent or cause symptoms. This article discusses the most common acquired benign and malignant vascular tumors, with an emphasis on their evaluation and treatment.