Vagal Schwannoma Research Articles

Ancient cervical vagal schwannoma and an interposition great auricular to vagus nerve graft

Introduction: Schwannomas of the cervical vagus nerve are exceedingly rare and have a myriad of clinical presentations depending on the tumor size and its proximity to neurovascular structures. We report a rare case of a cervical vagal schwannoma that was successfully treated with en bloc resection and reconstruction with a great auricular to vagus nerve graft. Case Report: A 42-year-old male presented with an asymptomatic, right lateral neck mass. Magnetic resonance imaging revealed a lesion 2.4 cm × 1.4 cm × 4.0 cm in size, located between the carotid arteries and internal jugular vein displacing the internal carotid artery anteromedially. En bloc resection of the lesion and reconstruction of the vagus nerve with a great auricular interposition graft was performed. Conclusion: Vagal schwannomas are rare, benign neurogenic tumors. Complete surgical excision is the mainstay of treatment. When a safe surgical plane cannot be identified between the neurogenic tumor and the main nerve trunk, en bloc resection with reconstruction should be considered.

Comprehensive analysis of radiological and surgical predictors in cervical sympathetic schwannomas: a novel staging approach and its implications.

Vagal schwannomas are well-documented, but cervical sympathetic chain schwannomas (CSCS) are rare, with most knowledge from case reports. This study aims to identify radiological predictors of misdiagnosis and factors guiding surgical approaches based on tumor size and extent. An ambispective analysis was conducted on 21 cases of CSCS, examining preoperative data, intraoperative findings and the questionnaire to identify the potential predictors. Tumors were classified into three types based on their relationship with the carotid sheath, and this classification was correlated with vessel ligation and postoperative neural outcomes. An excellent agreement was found between radiologist on new classification system(Kappa:0.89). Tumor classification revealed a diverse distribution, with 6 cases identified as Type 1, 6 as Type 2, 5 as Type 3, and 4 as Type 3S. The necessity of external carotid artery (ECA) ligation correlated with the tumor type. Type 3 tumors required ECA ligation in 50% of cases, while Type 1 and Type 2 tumors predominantly involved vascular preservation. Postoperative complications included vagal palsy in 28.5% of cases and first bite syndrome in 71.4%. Accurate preoperative planning and a novel staging system can enhance surgical outcomes and reduce postoperative complications as validated by our study.

Angiosarcoma Arising in a Vagal Schwannoma - Report and Literature Review.

Schwannoma and angiosarcoma are rare occurrences. Angiosarcoma's occurrence in a preexisting schwannoma is a very rare event with only fifteen cases reported in the literature. We report the sixteenth case of angiosarcoma arising in a schwannoma, a 30-year-old male patient with a long-standing (15 years) history of right neck lump. The lump measured around 10x7 cm at the time of presentation and all cranial nerves were intact. We performed a fiberoptic laryngoscopy and a computed tomography scan of the head and neck with contrast. Our provisional diagnosis was a parapharyngeal space neoplasm, most likely a salivary gland malignancy. The tumor was excised surgically. On histopathology it contained two distinct tissue architectures representing a schwannoma and epithelioid angiosarcoma, also confirmed on immunohistochemistry. Literature review of these limited cases implicates a poor prognosis of the disease. The pathogenesis is uncertain, but the theories put forward suggest chronic vascular stasis or vascular endothelial proliferation as possible etiologies. The main takeaway of our report is to consider the potential of malignancy in long standing cases of schwannomas. Prompt surgical treatment should be offered, and the patient and their family be counselled for postoperative adjuvant treatment for better prognosis.

Abstract 1085: Vagal Nerve Schwannoma Presenting As A Syncope In A Young Patient

Introduction: Vagal schwannomas are rare, benign masses that usually develop in the cervical region but can arise anywhere along the vagus nerve. Patients may be asymptomatic; however, in most cases, patients present in their third to sixth decades, with neck swelling, hoarseness or cough. Herein, we discuss a case report of a 26-year-old male who presented after an abrupt syncopal episode with sinus bradycardia and was diagnosed with vagal schwannoma. Case Presentation: A 26-year-old male with a past medical history of bipolar disorder was brought to the hospital after an abrupt syncopal episode while driving. He denied any palpitations or prodromal symptoms prior to loss of consciousness. Inpatient cardiac monitoring revealed persistent sinus bradycardia, without high degree AV block. Treadmill EKG stress test confirmed chronotropic competence. CT head did not reveal other etiology of syncope. CT chest revealed a 2.4 x 2.0 cm left superior mediastinal mass. Cardiothoracic surgery was consulted for concern of thoracic outlet mass/mediastinal mass. Robotic-assisted left thoracoscopy was performed for mediastinal mass excision, which revealed a left vagus nerve schwannoma on frozen section. Discussion: Most vagus nerve schwannomas have been found to arise within the cervical course of the vagus nerve. Intrathoracic vagal schwannomas are rare. These tumors are usually asymptomatic, however varied symptoms such as pain, dysphagia and shortness of breath have been reported. In this case presented, the tumor possibly contributed to the heightened vagal tone and neurogenic syncope (with vasovagal syncope). It is also notable that our patient's resting heart rate normalized following excision of the mass. Of note, with a high reported incidence of peri-perioperative vocal cord paralysis, surgical excision remains a challenging mainstay of treatment in symptomatic patients. Conclusion: Vagal neoplasms should be hence considered as a differential diagnosis in patients with otherwise unexplained bradycardia with history of syncope. Reference: Neurocardiogenic syncope; Carol Chen-Scarabelli, cardiovascular nurse practitioner and Tiziano M Scarabelli, associate professor of internal medicine. BMJ 2004 Aug 7; 329 (7461):336-341.

A Case of Cervical Vagal Schwannoma in Which Laryngeal Evaluation Was Difficult

A Case of Cervical Vagal Schwannoma in Which Laryngeal Evaluation Was Difficult

Mandibular swing approach for high parapharyngeal space schwannoma

Parapharyngeal space tumours are rare, comprising 0.5% of all head and neck neoplasms. Different surgical approaches to this space have been described based on various criteria. Here, we are presenting a case report of a 65-year-old woman with left vagal schwannoma splaying the left internal carotid artery and internal jugular vein superiorly and splaying the left internal carotid artery and external carotid artery inferiorly, with its upper edge about 0.7 cm caudal to skull base. Surgery by a combined trans-cervical and mandibular swing approach was adopted for this case. The tumour which was extending so close to the skull base was removed in toto with an intact capsule and the neurovascular structures like the carotids, internal jugular vein and the vagus were preserved through a trans-mandibular approach. Postoperatively patient had no neurovascular compromise. Histopathological examination revealed vagal schwannoma. Trans-mandibular approach may appear complex, but it offers excellent surgical field to keep the delicate surrounding structures intact, to reduce the risk of haemorrhagic and avoid neurological complications.

Analysis of Nonvestibular Cranial Nerve Schwannomas

Background and Aim: Cranial nerve (CN) schwannomas other than the eighth nerve schwannoma are called nonvestibular cranial nerve schwannomas (NVCNS). This study aims to analyze the clinical profile, distribution, and surgical outcome of the NVCNS. Methods and Materials/Patients: This is a retrospective study analyzing the NVCNS conducted between January 2007 and December 2021. VIIIth cranial nerve schwannomas and conservatively managed NVCNS were excluded from the study. Results: This study included 25 patients with NVCNS. Ten patients (40%) had trigeminal schwannomas, 3(12%) facial nerve schwannomas, 9 (36%) lower cranial nerve (LCN) schwannomas, 1 (4%) vagal schwannoma in neck and 2(8%) hypoglossal schwannomas. Eight patients (32%) were male and 17(68%) were female. The age of the patients ranges from 13 to 62 years (mean 39 years +/- 14 SD). Findings of cerebellar involvement, VIIIth CN involvement, and headache are the most common clinical features. The posterior cranial fossa is the most common location of the tumors. Thirteen patients (52%) underwent gross total resection (GTR), 7(28%) underwent neartotal resection (NTR) and 5(20%) underwent subtotal resection (STR). All the patients improved in the postoperative period. Facial nerve palsy is the most common cranial nerve palsy occurring in the postoperative period. Conclusion: Trigeminal and lower cranial nerve (LCN) schwannomas are the most common NVCNS. The retrosigmoid suboccipital approach is the most commonly used surgical approach for tumors located in the posterior cranial fossa. Since NVCNS are benign lesions, postoperative clinical improvement along with decreased complications should be the goal of the surgery. Hence, gross total resection is the most commonly achieved extent of resection, near-total or subtotal resection can be done wherever gross total resection (GTR) is not possible in NVCNS.

Iatrogenic inner ear dehiscence associated with lateral skull base surgery: a systematic analysis of drilling injuries and their causal factors.

Drilling injuries of the inner ear are an underreported complication of lateral skull base (LSB) surgery. Inner ear breaches can cause hearing loss, vestibular dysfunction, and third window phenomenon. This study aims to elucidate primary factors causing iatrogenic inner ear dehiscences (IED) in 9 patients who presented to a tertiary care center with postoperative symptoms of IED following LSB surgery for vestibular schwannoma, endolymphatic sac tumor, Meniere's disease, paraganglioma jugulare, and vagal schwannoma. Utilizing 3D Slicer image processing software, geometric and volumetric analysis was applied to both preoperative and postoperative imaging to identify causal factors iatrogenic inner ear breaches. Segmentation analyses, craniotomy analyses, and drilling trajectory analyses were performed. Cases of retrosigmoid approaches for vestibular schwannoma resection were compared to matched controls. Excessive lateral drilling and breach of a single inner ear structure occurred in 3 cases undergoing transjugular (n=2) and transmastoid (n=1) approaches. Inadequate drilling trajectory breaching ≥1 inner ear structure occurred in 6 cases undergoing retrosigmoid (n=4), transmastoid (n=1), and middle cranial fossa approaches (n=1). In retrosigmoid approaches the 2-cm visualization window and craniotomy limits did not provide drilling angles to the entire tumor without causing IED in comparison to matched controls. Inappropriate drill depth, errant lateral drilling, inadequate drill trajectory, or a combination of these led to iatrogenic IED. Image-based segmentation, individualized 3D anatomical model generation, and geometric and volumetric analyses can optimize operative plans and possibly reduce inner ear breaches from lateral skull base surgery.

VAGAL AND CERVICAL SYMPATHETIC CHAIN SCHWANNOMA AND THEIR ANAESTHETIC CONCERNS

Schwannoma of head and neck is a rare tumor arising from neural component . Majority of patients with cervical vagal schwannoma present with mass in the lateral part of neck without any neurological deficit. Complete surgical resection is the treatment of choice. We report here a symptomatic cervical vagal schwannoma in a 46 year old female presenting with dyphagia for 2 weeks . MRI neck revealed a well defined smooth marginated non infiltrative lesion of size 19*23*35 mm, anteriorly abutting sternocleidomastoid and posteriorly abutting cervical segment of left vertebral arterycervical/ vagal origin . The patient was first planned for temporary cardiac pacing to avoid the consequences of vagal nerve handling like bradycardia intraoperatively. Standard GA was given. Superficial cervical plexus was given and the surgical area was anaesthetized to prevent the complications of vagal nerve handling. The tumor was dissected carefully and sent for biopsy. Patient reversed and intraoperative was uneventful. After excision of tumor, our patient complained of mild left eyelid drooping indicating the tumor excised to be of cervical sympathetic chain origin

A rare case of schwannoma of the mandibular nerve extending to parapharyngeal space managed with a preauricular trans mandibular approach

Schwannoma also referred to as neurilemmoma or neurinomas arise from the Schwann cells of the autonomic nerves, peripheral nerves and cranial nerves except optic and olfactory nerves. Head and neck schwannomas account for 25 to 45% of schwannomas and the most common being vagal schwannomas and cervical sympathetic chain schwannomas. Schwannomas arising from the trigeminal nerve are rare and this case report consists of a schwannoma presenting as a left preauricular swelling involving the region of parotid extending from foramen ovale through the infratemporal fossa up to the parapharyngeal space. A combined preauricular trans mandibular approach enabled a complete excision of the tumor. Histopathological confirmation of a benign schwannoma was obtained and the patient is feeling well with minimal postoperative facial palsy with no trismus, occlusion abnormalities and no signs of recurrence on follow up.

Symptoms: Dizziness and Deafness

Symptoms: Dizziness and Deafness

Managing Multiple Schwannomatosis of Vagus and Hypoglossal Nerves: The Unanticipated Complications.

Schwannomas are rare benign tumours of the nerve sheath. Extracranial schwannomas are usually found solitary, in the absence of other features of neurofibromatosis. The non NF1/2 multiple schwannomas are collectively described as the clinical scenario called schwannomatosis. We present a rare case of two extracranial schwannomas involving vagus and hypoglossal nerves in a 22-year-old female along with an unusual surgical complication of CSF leak presenting as post-op neck mass and a review of previously reported similar cases. A thorough literature search using the MeSH terms-'schwannomatosis' or 'Multiple Schwannoma' and 'Vagal Schwannoma' and 'Hypoglossal Schwannoma', was conducted using online databases and augmentated by hand search. A total of 13 reported cases were found and reviewed. Of the 14 cases described from review of literature and our case, 50% had involvement of vagus nerve as one of the components. 12.5% had hypoglossal and cervical sympathetic plexus involvement each. All patients underwent surgical excision. Half of the patients suffered intraoperative nerve sacrifice with resultant severe functional deficits like vocal cord paralysis, Horners syndrome, hypoglossal palsy, facial palsy and eleventh nerve palsy. In our case, there was un-unusual complication of CSF leak presenting as a cervical neck mass. Multiple extracranial head and neck schwannomas are rare and their management is fraught with several complications. Decision to operate is often perplexing. However, early planned surgical excision and preservation of nerve of origin minimizes the impact on quality of life postoperatively.

A Misdiagnosed Cervical Vagal Schwannoma: A Case Report and Review of Its Imaging Features and Differential Diagnosis

A Misdiagnosed Cervical Vagal Schwannoma: A Case Report and Review of Its Imaging Features and Differential Diagnosis

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

BackgroundSchwannomas 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 presentationA 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.ConclusionsThis 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.

Neurofibroma at an Aberrant Location - A Case Report

Background: Neurofibromas are benign tumors of peripheral nerve sheath and are uncommon in cervical part of vagus nerve. A 35-year female presented with nontender swelling on the left side of the neck since 2 months with no family history. Clinical diagnosis was made as? carotid body tumor/parapharyngeal mass. CT neck showed well defined hypodense oval mass in the left upper neck in the carotid space reported as features suggestive of schwannoma. Post-operative diagnosis of left vagal schwannoma was made and sent for histopathological examination. Gross morphology was well circumscribed grey white mass measuring 6x4x3cms with gelatinous cut surface. On microscopy it was neoplasm composed of spindle cells with poorly defined cell borders having buckled nuclei with pale eosinophilic cytoplasm. These cells are arranged loosely in the background of collagenous matrix. No evidence of atypia or malignancy noted. Features consistent with Neurofibroma. Conclusion: Histopathological confirmation is essential for the diagnosis of neurofibroma in cervical part of vagus nerve as it is an uncommon site for the lesion to develop. Keywords: Neurofibroma, Cervical part of vagus nerve, Aberrant site

Intracapsular enucleation of cervical schwannomas via retroauricular hairline incision

IntroductionCervical schwannomas are rare diseases treated by complete resection, leading to potential neurological deficits. Intracapsular enucleation is an acceptable alternative to preserve the involved nerve function, but the clinical outcomes by an aesthetically pleasing approach remain unrevealed. This study evaluated functional and oncological outcomes after intracapsular enucleation of cervical schwannomas via retroauricular hairline (Roh's) incision. Materials and methodsThis prospective observational study included 17 patients with cervical schwannomas who underwent intracapsular enucleation to preserve the involved nerve and capsule via Roh's incision. Each patient was evaluated with any complications, nerve function, subjective satisfaction, voice and swallowing questionnaires, and local recurrence. ResultsIntracapsular enucleation via the Roh's incision was successfully applied to all patients for a median operation time of 48 min. Tumours of 4.5-cm median size were removed with the minimization of injury to the nerve and sheath deflected on the surface of the tumour. Temporary and permanent paralysis of the unilateral vocal fold was found in 3 (33%) and none of 9 patients with vagal schwannomas, respectively. Horner's syndrome occurred temporarily in two of 5 (40%) patients with sympathetic chain schwannomas. Postoperative complications were minor. Subjective satisfaction of scar and facial deformity was high, and subjective voice and swallowing difficulties were minimal one year after surgery. No recurrence was found in the patients for a median follow-up of 82 months. ConclusionIntracapsular enucleation via Roh's incision is a viable treatment for cervical schwannomas with the highest functional, cosmetic, and oncological outcomes.

Extracranial Head and Neck Schwannomas: A Case Series and Review of Literature.

Schwannomas are benign nerve tumors of schwann cell origin. Schwann cells are derived from neural crest and are therefore of neuroectodermal origin. 25-40% of all schwannomas arise in the head and neck region. The most common schwannomas are vagal schwannomas and cervical schwannomas in the head and neck region. Here we present two cases of head and neck schwannomas which were not identified as originating from any major nerve. The first patient had a schwannoma in the infratemporal region which was excised by performing transzygomatic approach, and the second patient had a schwannoma in the left lateral part of the neck which was excised using a cervical crease incision.

A rare lesion of the wanderer’s nerve with an unusual postoperative complication: vagal nerve schwanomma

<p class="abstract">Vagal nerve schwannomas are rare parapharyngeal tumours which was first described by Figi in 1933. Hoarseness is the most common symptom. Occasionally, a paroxysmal cough may be produced on palpating the mass. This is a clinical sign, unique to vagal schwannoma. Presence of this sign, along with a mass located on the medial border of the sternocleidomastoid muscle, should make clinicians suspicious of vagal nerve sheath tumours. However, in our case, the patient presented to us with a swelling in the neck and no other symptoms. MRI in the pre-operative work-up is helpful in defining diagnosis and in evaluating the extent and the relationship. A careful intracapsular enucleation with nerve-sparing technique remains the treatment of choice in order to avoid nerve injury which can lead to vocal cord paralysis. Horner’s syndrome following cervical surgeries is a rare phenomenon. Its occurrence following excision of a vagal schwannoma can occur due to direct injury to cervical sympathetic chain during excision or indirect injury due to traction on the sympathetic chain. Here is a case report of a patient diagnosed with left vagal scwanomma who developed horner’s syndrome post operatively.</p>

THAT NAGGING COUGH: INTRATHORACIC SCHWANNOMA WITH HORNERS SYNDROME

SESSION TITLE: Medical Student/Resident Lung Pathology SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Malignant tumors in the mediastinum are unusual, accounting for less than 1% of mediastinal tumors. Of those, neural origin are usually benign in the mediastinum [1]. The most common tumors are located in the posterior mediastinum and are neurogenic such as schwannomas but also may be pleural fibromas. Definitive diagnosis requires tissue sampling [2]. Incidence is extremely rare, with 6% of giant schwannomas originating from the vagus nerve. Currently there are not enough cases to have a prevalence. Horner Syndrome is associated with mediastinal tumors, which is characterized by obstruction of the sympathetic pathway to the eye and face consisting of ipsilateral myosis, partial proptosis and anhidrosis [3]. CASE PRESENTATION: Thirty one-year-old female with a past medical history of two miscarriages was seen in the office for an upper respiratory infection and prescribed antibiotics and ordered a two view chest x-ray which showed a 6 cm round mass in the right upper lobe suspicious for pancoast tumor [Figure 1A, B]. She had been complaining of headaches, right eyelid dropping, dizziness and uneven pupils as her right side was smaller than left. Subsequently had computed tomography (CT) chest showing small right apical mass with fibrous tumor of the pleura with involvement possibly of the nerve sheath measuring 6 cm x 4.8 cm x 5.7 cm [Figure 2A, B]. Patient had CT guided lung biopsy which demonstrated S100, BCL-2 and CD 99+ compatible with schwannoma [Figure 3A-D]. She had positron emission tomography (PET) scan performed which showed intense fluorodeoxyglucose (FDG) uptake in the right apical region with standardized uptake values (SUV) max of 6.7 with no FDG avid thoracic lymphadenopathy, extensive FDG uptake within hypermetabolic fat in the left neck, supraclavicular fossa, and superior right suprarenal fat. Focal FDG uptake localizing to the posterior left neck with SUV max of 3.8. Patient was referred to surgery where she had brachial plexus exploration and dissection of thoracic inlet tumor resected through VATS. DISCUSSION: Neurogenic tumors are the most common type of neoplasia the posterior mediastinum. This consists of schwannomas and neurofibromas along with ganglion neuroblastomas. Recognition of adjacent nerves as well are well-defined margins in the presence of split fat sign may suggest benign tumor [4]. This patient developed a rare association of intrathoracic schwannoma that presented itself as Horner syndrome. Although Horner syndrome is a well-known association of sympathetic chain schwannomas of the neck and malignant lung tumors, it is uncommon for it to be presented as a benign intrathoracic lesion. Treatment is surgical resection. CONCLUSIONS: Our patient had a successful brachial plexus exploration and dissection of thoracic inlet tumor resected through VATS. Afterwards she had no more signs of Horner's syndrome. Reference #1: Angelopoulos, E., Eleftheriou, K., Kyriakopoulos, G., Athanassiadi, K., Rontogianni, D. and Routsi, C. (2016). A Giant Intrathoracic Malignant Schwannoma Causing Respiratory Failure in a Patient without von Recklinghausen’s Disease. Case Reports in Medicine, 2016, pp.1-3. Reference #2: Ritz, R., Pehl, A., Bartsch, D. and Kirschbaum, A. (2013). Giant Intrathoracic Left-Sided Vagal Schwannoma. The Thoracic and Cardiovascular Surgeon Reports, 02(01), pp.019-022. doi: 10.1055/s-0033-1337368 Reference #3: Torpiano, P., Borg, E., Cassar, P. and Manche', A. (2013). Intrathoracic schwannoma with Horner syndrome. Case Reports, 2013(dec06 1), doi: bcr2013201247. DISCLOSURES: No relevant relationships by Apurwa Karki, source=Web Response No relevant relationships by Salman Khan, source=Web Response No relevant relationships by Nathaniel McElhaney, source=Web Response No relevant relationships by Mahin Rehman, source=Web Response No relevant relationships by FARAZ Siddiqui, source=Web Response

Lateral Suboccipital Approach in Semisitting Position for Resection of Vagal Schwannoma: 2-Dimensional Operative Video.

We present a case of a sizeable vagal schwannoma that was resected through a lateral suboccipital approach in semisitting position. An extra-axial lesion, occupying the left cerebellomedullary cistern and extending from the pontomedullary junction to the jugular foramen was incidentally discovered in a 40-year-old woman afflicted with secondary progressive multiple sclerosis during repeated magnetic resonance imaging ( Fig. 1 ). On physical examination, a mild deviation of the uvula to the right and a diminished gag reflex were observed. The patient was referred to our department after considerable growth of the lesion was noted and a broad interdisciplinary consensus was reached to treat the lesion surgically. A gross total resection was achieved, histopathology confirmed a WHO I schwannoma with a low proliferation index. Postoperative dysphonia resolved completely within a few weeks, there was no collateral neurological deficit and especially no functional dysphagia. At 3-year follow-up, there was no indication of residual or recurrence. This 2-dimensional video demonstrates pre- and postoperative imaging, positioning and set-up of operating room, anatomical and surgical nuances of the skull base approach, and the operative technique for microdissection of the schwannoma from the critical neurovascular structures ( Fig. 2 ). In summary, the lateral suboccipital approach in semisitting position is a powerful tool in the armamentarium for the microsurgical management of various pathologies residing in the posterior cranial fossa, especially large and vascularized schwannomas. Provided the necessary anesthesiological precautions and intraoperative procedures the semisitting position is safe and effective. The link to the video can be found at: https://youtu.be/-9o_qJGkQhg .