Asymptomatic Purely Intracranial Vagal Schwannoma: Clinical Case Report and Literature Review.

Vagus nerve schwannoma is an infrequently occurring schwannoma, in which a distinct subtype exists wherein the tumor is confined to the cerebellomedullary cistern without invading the jugular foramen. This unique tumor is called purely intracranial vagal schwannoma. In this case report, we present a case of purely intracranial vagal schwannoma in its asymptomatic early phase, incidentally discovered during surgery performed on a patient with hemifacial spasm. Because of the small size of the tumor, we definitively recognized that it originated from the second rootlet on the caudal side. The tumor was totally resected uneventfully and a favorable prognosis was achieved. Furthermore, we conducted a comprehensive literature review to summarize the classification, origin, and surgical complications associated with this rare tumor type. Based on our literature review, we propose that: 1) the origin of tumor is related to the time of onset of symptoms, 2) nearly all purely intracranial vagal schwannomas can be entirely resected and favorable prognosis can be achieved, and 3) surgeons should be aware of potential cardiovascular complications during surgical procedures.

Delayed vagal nerve compressive neuropathy following placement of vagal nerve stimulator: case report.

Vagal neuropathy causing vocal fold palsy is an uncommon complication of vagal nerve stimulator (VNS) placement. It may be associated with intraoperative nerve injury or with device stimulation. Here we present the first case of delayed, compressive vagal neuropathy associated with VNS coil placement which presented with progressive hoarseness and vocal cord paralysis. Coil removal and vagal neurolysis was performed to relieve the compression. Larger 3 mm VNS coils were placed for continuation of therapy. Coils with a larger inner diameter should be employed where possible to prevent this complication. The frequency of VNS-associated vagal nerve compression may warrant further investigation.

Functional vagal paraganglioma developing 15 years after resection of a retroperitoneal paraganglioma.

The patient, a 40-year-old woman, was diagnosed as having a functional right vagal paraganglioma (PGL) 15 years after undergoing resection for a retroperitoneal PGL. 123I-MIBG scintigraphy showed no accumulation, but as the blood noradrenaline and urinary normetanephrine concentrations were elevated, the tumor was judged as being functional, and surgery was scheduled. The patient was started on doxazosin infusion and embolization of the tumor feeding vessel was performed before the surgery. Intraoperative examination showed that the tumor was contiguous with the vagal nerve, necessitating combined resection of the vagal nerve with the tumor. Postoperatively, the catecholamine levels returned to normal range. Histopathologically, the tumor was diagnosed as a moderately differentiated, intermediate-malignant-grade PGL, with a GAPP score of 4 to 6. No non-chromaffin tissue was observed in the tumor background, so that the functional vagal PGL was considered as a sporadic metachronous tumor rather than as a metastasis from the retroperitoneal PGL. More than half of head and neck paragangliomas (HNPGLs) are reported to arise in the carotid body, and about 5% from the vagal nerve. In addition, HNPGLs rarely produce catecholamines. Herein, we consider the relationship with the previously resected retroperitoneal PGL based on a review of the literature.

Report of a vagal paraganglioma at the cervicothoracic junction.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: We describe an unusual case of vagal paraganglioma at the cervicothoracic junction with retrosternal extension requiring a sternotomy for surgical excision.

Cervical vagal nerve schwannoma induced arrhythmia: a rare case report and literature review.

BACKGROUND: Schwannomas are benign tumors deriving from the sheath of cranial and peripheral nerves. The vagus nerve is comprised of a complex neuro-endocrine-immune network that maintains homeostasis, most tracts of it play a role in parasympathetic activity. We present an example of a rare cervical vagal schwannoma case accompanied by arrhythmia. CASE PRESENTATION: A 35-year-old female patient with a left cervical vagus schwannoma and ventricular arrhythmia underwent schwannoma resection in the operating room. The patient's suppressed heart rate increased after tumor removal, and the cardiac rhythm returned to normal postoperatively. Pathological examination demonstrated the diagnosis of schwannoma. CONCLUSIONS: This case explains the link between the vagus nerve and the cardiovascular system, proving that a damaged cervical vagus nerve can inhibit the heart rate and lead to arrhythmias, and eventually requiring surgical intervention.

Left Paraglossal Approach for Airway Management of Patient With Large Neck Mass in an Austere Environment: A Case Report.

We present the case of a 25-year-old man with a significant neck mass and describe the technique used for managing his airway given limited equipment in an austere environment. Physical examination and imaging revealed significant proximal airway involvement. Without access to a fiberoptic bronchoscope, we sought a technique that might avoid contact with the mass from both the laryngoscope and endotracheal tube. In this case report, we describe our approach to left paraglossal laryngoscopy and intubation under general anesthesia-a method not well described in the literature and proved to be imperative for our airway management.

Intraoperative mapping and monitoring of sensory vagal fibers during vagal schwannoma resection.

Vagal schwannomas are rare, benign tumors. Intermittent intraoperative neuromonitoring via selective stimulation of splayed motor fibers running on the schwannoma surface to elicit a compound muscle action potential has been previously reported as a method of preserving vagal motor fibers. In this case report, vagal sensory fibers are mapped and continuously monitored intraoperatively during high vagus schwannoma resection using the laryngeal adductor reflex (LAR). Mapping of nerve fibers on the schwannoma surface enabled identification of sensory fibers. Continuous LAR monitoring during schwannoma subcapsular microsurgical dissection enabled sensory (and motor) vagal fibers to be monitored in real time with excellent postoperative functional outcomes. Laryngoscope, 129:E434-E436, 2019.

A novel approach to neurologic function sparing surgical management of vagal schwannomas: Continuous intraoperative nerve monitoring of the laryngeal adductor reflex.

BACKGROUND: Vagal schwannomas are rare, benign tumors of the head and neck. Nerve damage during surgical resection is associated with significant morbidity. A new technique of continuous intraoperative nerve monitoring (IONM) that allows for real-time intraoperative feedback has recently been used for thyroid and cervical spine surgeries but has not previously been used in vagal schwannoma surgery. METHODS: Case series of three patients who underwent vagal schwannoma excision utilizing this novel IONM technique. The recurrent laryngeal and vagus nerves were monitored via the laryngeal adductor reflex (LAR) using an electromyographic endotracheal tube. RESULTS: Three patients with suspected vagal schwannomas were treated surgically using the intracapsular enucleation approach with a combination of intermittent IONM and continuous IONM of the LAR. CONCLUSION: This combination of continuous and intermittent IONM can be used to preserve vagal laryngeal innervation and function and may represent the future standard of care for vagal schwannoma excision.

Vagal schwannomas of the head and neck: A comprehensive review and a novel approach to preserving vocal cord innervation and function.

BACKGROUND: Schwannomas, benign tumors arising from neurolemmocytes, are the most common type of peripheral nerve tumors. Extracranial schwannomas are most often found in the parapharyngeal space, commonly involving the vagus nerve to cervical sympathetic trunk. Vagal schwannomas present several unique clinical and therapeutic challenges. METHODS: A comprehensive literature review was conducted on 197 articles reporting 235 cases of cervical vagal schwannomas. Presenting symptoms, treatment approach, and postoperative outcomes were recorded and analyzed. RESULTS: Vagal schwannomas commonly present as asymptomatic neck masses. When they become symptomatic, surgical resection is the standard of care. Gross total resection is associated with higher postoperative morbidity compared to subtotal resection. Initial reports using intraoperative nerve monitoring have shown improved nerve preservation. Recurrence rates are low. CONCLUSION: The combination of intermittent nerve mapping with novel continuous vagal nerve monitoring techniques may reduce postoperative morbidity and could represent the future standard of care for vagal schwannoma treatment.

Purely intracranial vagal schwannoma: A case report of a rare lesion.

We present a rare intracranial vagal schwannoma along with its preoperative, intraoperative, and postoperative course.

Diagnosis and management of pediatric cervical vagal schwannoma.

Schwannomas arising from the vagus nerve are extremely rare in children, with only 15 cases reported in the world literature. We describe a pediatric case of cervical vagal nerve schwannoma successfully treated with cranial nerve-sparing surgery. Our patient presented extensive mass in the right side of the neck with ipsilateral Horner's syndrome. Her first sign, anisocoria, was diagnosed at the age of 1.5 y, making her the youngest vagal schwannoma case ever reported. Using an ultrasonic surgical aspirator and nerve monitoring, a multidisciplinary team successfully removed the mass with no recurrence after 2 years of follow-up.

Polymyalgia rheumatica and vagal paraganglioma.

INTRODUCTION: Vagal paraganglioma are rare tumors that are mostly asymptomatic. We report a case of vagal paraganglioma associated with paraneoplastic polymyalgia rheumatica and review the literature on benign paragangliomas of the head and neck associated with paraneoplastic syndrome. CASE REPORT: A 53-year-old man presented with atypical polymyalgia rheumatica. MRI revealed a tumor that was then surgically excised. Histological examination confirmed the diagnosis of benign vagal paraganglioma. Rapid, complete and permanent resolution of all rheumatological symptoms were observed postoperatively, confirming the diagnosis of paraneoplastic polymyalgia rheumatica. CONCLUSION: Paraganglioma of the neck associated with paraneoplastic syndrome remains exceptional. A predisposing gene mutation must be systematically investigated. Long-term surveillance must be ensured due to the risk of local recurrence, second tumors or metastasis.

Extracranial schwannoma in the carotid space: A retrospective review of 91 cases.

BACKGROUND: Schwannomas of the vagus nerve and cervical sympathetic nerve are rare; hence, only limited information exists regarding their diagnosis and clinical management. METHODS: We conducted a retrospective review of the clinical features, imaging studies, and treatment results of patients with schwannoma of the vagus nerve and schwannoma of the sympathetic nerve. RESULTS: Of 91 patients, 91% (n = 83) were preoperatively diagnosed with schwannoma tumors. Using the hyoid bone as an anatomic landmark, the location of the schwannoma of the vagus nerve in the carotid space was significantly different to the location of schwannoma of the sympathetic nerve (p = .003). Although 52 of the 76 patients followed up (68%) had postoperative nerve weaknesses, 13 patients (50%) and 14 patients (53.8%), respectively, fully recovered from schwannoma of the vagus nerve and schwannoma of the sympathetic nerve. CONCLUSION: In the carotid space, schwannomas of the vagus nerve are usually located below the hyoid bone, whereas schwannomas of the sympathetic nerve more commonly arise from the suprahyoid compartment. Accurate preoperative diagnosis and the intracapsular enucleation surgical approach decreased the incidence of postoperative morbidity. © 2016 Wiley Periodicals, Head Neck 39: 42-47, 2017.

[Synchronous unilateral vagal paraganglioma and carotid body tumor:one case report].

One case of synchronous unilateral vagal paraganglioma and carotid body tumor was reported. The preoperative medical imaging revealed no obvious abnormalities at the carotid bifurcation. In addition to the bigger vagal paragaglioma，a smaller carotid body tumor was discovered during operation.

Vagoglossopharyngeal neuralgia revealed through predominant digestive vagal manifestations. Case report and literature review.

Vagoglossopharyngeal neuralgia is a rare pathology whose atypical forms, dominated by syncopal manifestations, are still rarer. Although the territory of the vagus nerve involves, beyond the cardiovascular system, the respiratory and the digestive systems, there is no report in literature of atypical forms other than syncopal. Therefore, the authors were prompted to report the case of a patient whose vagoglossopharyngeal neuralgia was predominantly revealed by digestive symptoms. A 58-year-old patient presented with stereotypical severe digestive disturbances including nausea, vomiting and diarrhoea. High definition cranial MRI showed a neurovascular conflict between the posterior inferior cerebellar artery and the IXth and Xth nerves, on the right side. A microsurgical decompression was carried out which confirmed the vascular compression and successful transposition of the artery. One year after the surgery, the patient was free from all painful and digestive symptoms. A survey of the literature did not find any reference to digestive symptoms together with the neuralgia; only a syncopal type of cardiac symptoms related to the parasympathetic nervous system were described. The hypothesis was that the revealing digestive symptoms are linked to a similar parasympathetic mechanism, implying the visceral component of the Xth cranial nerve.

Superiority of resection over enucleation for schwannomas of the cervical vagus nerve: A retrospective cohort study of 22 consecutive patients.

INTRODUCTION: Schwannoma of the cervical vagus nerve is rare. Treatment options include intracapsular enucleation and en bloc resection. The purpose of this study was to compare the outcomes of enucleation and resection in terms of postoperative mortality and morbidity, freedom from vocal cord palsy, freedom from local recurrence, quality-adjusted life-year (QALY) and vocal handicap index (VHI). METHODS: Twentytwo consecutive patients were divided into two groups. Patients in group A (n = 9) underwent intracapsular enucleation, whereas patients in Group B (n = 13) underwent en bloc resection. Main endpoints of the study were postoperative mortality and morbidity, freedom from vocal cord palsy, freedom from local recurrence and quality of life. The quality of life after surgery was assessed according to the quality-adjusted life-year (QALY) EQ-5D-5L methodology, and calculation of the voice handicap index (VHI). RESULTS: Postoperative mortality was nil. Morbidity included 1 wound dehiscence in group A and 2 transitory dysphagias in group B. Freedom from vocal cord palsy was 22% in group A and zero in group B (p = 0.15). Operation-specific local recurrence rate was 33% (3/9 patients) in group A and nil in group B (0/23 patients) (p = 0.05). QALYs was 0.55 in group A and 0.54 in group B (p = 1.0). VHI was 23.77 in group A and 26.15 in group B (p = 1.00). CONCLUSION: Resection is superior to enucleation in terms of freedom from local recurrence. Functional results are comparable for both techniques.

Ganglioneuromas involving the hypoglossal nerve and the vagus nerve in a child: Surgical difficulties.

Ganglioneuromas are benign tumors that arise from the Schwann cells of the autonomic nervous system. They are usually seen in the posterior mediastinum and the paraspinal retroperitoneum in relation to the sympathetic chain. In the head and neck, they are usually related to the cervical sympathetic ganglia or to the ganglion nodosum of the vagus nerve or the hypoglossal nerve. We describe what we believe is the first reported case of multiple ganglioneuromas of the parapharyngeal space in which two separate cranial nerves were involved. The patient was a 10-year-old girl who presented with a 2-year history of a painless and slowly progressive swelling on the left side of her neck and a 1-year history hoarseness. She had no history of relevant trauma or surgery. Intraoperatively, we found two tumors in the left parapharyngeal space-one that had arisen from the hypoglossal nerve and the other from the vagus nerve. Both ganglioneuromas were surgically removed, but the affected nerves had to be sacrificed. Postoperatively, the patient exhibited hypoglossal nerve and vocal fold palsy, but she was asymptomatic. In addition to the case description, we discuss the difficulties we faced during surgical excision.

[Cystic mediastinal schwannoma appended to the vagus nerve]. / Schwannomes kystiques du médiastin développés au dépend du nerf vague.

INTRODUCTION: Schwannomas are a form of rare tumor, arising from neural tissue and representing 2 % of mediastinal tumors. They are usually located in the posterior mediastinum, most often in the paravertebral gutters and typically appended to intercostal nerves. CASE REPORTS: We report two cases of unusual mediastinal schwannomas, appended to the vagus nerve. The schwannoma was located in the subcarinal region in the first case and in the right para-tracheal region in the second case. The lesions were thought to be bronchogenic cysts preoperatively in both cases because of a cystic appearance on preoperative CT scan and endobronchial ultrasonography. A surgical approach was adopted to remove the tumors. Video-assisted thoracoscopy was used in one case and robotic-assisted surgery in the second case, without any complication, allowing for complete resection and to establish a certain pathological diagnosis. CONCLUSION: Despite this location and cystic presentation being unusual, schwannoma should be considered as a possible cause of cystic lesions in the mediastinum. Minimally invasive surgery allows for complete resection and definitive pathological diagnosis.