An association between essential hypertension and neurovascular compression of the rostral ventrolateral medulla has been reported. Vagal schwannoma involving esophagus the annals of thoracic. Schwannomas are benign nerve sheath neoplasms arising from peripheral or cranial nerves. I ntr aopera tive image of the schwan noma a the stoma ch, bthe vagal. Vagal refers to the vagus nerve which is the 10th cranial nerve. Metastatic papillary carcinoma of the thyroid may also enhance with contrast. However, surgery is not always recommended because of the indolent nature of the tumor and the risk of postoperative neural deficits. Schwannoma can occur anywhere in the gi tract but is most common in the stomach, followed by the colon and rectum. The rate of nerve palsy at 6 months after complete tumor resection and intracapsular enucleation was 100 1111 and 31% 516, respectively.
Mayo clinic surgeons carefully remove your schwannoma while taking care to preserve nerve fascicles that arent affected by your tumor. More serious causes, such as meniere disease or vestibular schwan noma, can be excluded during the evaluation. Kissing neurinomas of the vestibular and vagal nerves. Such type of association is unique and new to voluminous medical literature. B internal and external carotid arteries left carotid body tumor splaying of the angiogram of a by. Hypoglossal nerve schwannoma in the submandibular region.
Schwannoma originating from the cervical vagus nerve is an extremely rare neoplasm. Benign posterior mediastinal schwannomamultiple diagnostic. The operation was performed by the transcervical approach. Differentiation between schwannoma of the vagus nerve and. Compression of the rostral ventrolateral medulla by a. These tumors are typically benign and slow growing. A case report med j malaysia vol 67 no 3 june 2012 343 was due to medialization of the right lateral pharyngeal wall secondary to parapharyngeal involvement. Mr evaluation typically shows masses of intermediate signal on t1weighted images and increased signal intensity on t2weighted images with smooth, welldelineated margins and a homogeneous overall appearance. In some cases, the history and the age of patients can render diagnoses incorrect. As the authors had just experienced only one case of the schwan noma of sympathetic origin, the followup studies are needed for the accuracy of this study.
A schwannoma shwonoma is a tumour of the tissue that covers nerves, called the nerve sheath. It is usually asymptomatic and presents as a painless, palpable mass in the neck. Such a lesion may be considered as a caveat to the paradigm of. They are one of the two most common intradural extramedullary spinal tu. Ct and mri evaluation of nerve sheath tumors of the cervical vagus nerve. Vagal nerve schwannoma usually occurs between the third and fi fth decades of life, it does not show sex predilection both sexes being equally affected and it most often presents as a painless, slowgrowing, lateral neck mass. Chronic progressive dysphagia was the only complaint related to the schwannoma. They are usually slowgrowing benign lesions closely associated with the vagus nerve.
Ct and mri evaluation of nerve sheath tumors of the. In treating schwannoma patients, it is critical to determine the origin of the tumor to preserve nerve function. This nerve is the only nerve that runs from the brain all the way down through the body. Case report a cervical vagal schwannoma mimicking a. Schwannoma is a benign tumor that originates from the neural sheath of schwann cells. Vagal schwannoma is an extremely rare tumor that occurs along the path of the vagus nerve cn x having its location posterior to the carotid bifurcation the vagus nerve runs along the posterior aspect of the carotid sheath the main differential diagnosis at such location is the vagal paraganglioma, however, schwannoma shows moderate enhancement while paraganglioma shows. Single case report schwannomas of the cervical vagal nerve are rare neoplasms, usually occurring between the third and the sixth decade of life. Functional nerve preservation in extracranial head and. In the parapharyngeal space, schwannomas most commonly arise from the vagus nerve and cervical sympathetic chain. I know it has something to do with opening up your skull because the vagus nerve comes from your brain, so they would have to go up and find it.
It was suggested that paroxysmal cough during fnac or palpation of the mass was diagnostic for the vagal nerve schwannoma. They dont demonstrate any sex predilection, and they often present as slowgrowing, palpable neck masses, left. Hypoglossal and accessory nerve preservation is also necessary to prevent postoperative dysphagia. The tumor cells always stay on the outside of the nerve, but the tumor itself may either push the nerve aside andor. Case 1 is a schwan noma of the vagus nerve in the parapharyngeal space. Vagal schwannoma is a rare paraganglioma of head and neck and the best treatment is still controversial. The eighth cranial nerve is the most susceptible to schwannomas. One case report of a schwannoma involving the sympathetic plexus in the carotid canal has been reported previously. Diagnostic approach to patients with tinnitus kenneth s. Case reports five cases of nf2 were seen at kansai medical university hospital between 1992 and 1996. A case of mediastinal malignant epithelioid schwan noma mes is reported. We report the case of patient diagnosed with intrathoracic vagus nerve schwannoma. They may be intracranial only or have both intra and extracranial extension. Cervical vagal schwannoma is a benign, slowgrowing mass, often asymptomatic, with a very low lifetime risk of malignant transformation in general population, but diagnosis is still a challenge.
We evaluated the validity of preoperative imaging studies in distinguishing the neurological origin of the schwannomas of the head and neck, and the efficacy of intracapsular enucleation in preserving nerve function. Pdf schwannoma in the head and neck is usually arising from parapharyngeal space, but masseteric schwannoma is very rare. Nov 26, 2009 schwannoma originating from the vagus nerve within the mediastinum is a rare, usually benign tumor. Clinically, schwannomas present as asymptomatic, slowly enlarging lateral neck. Keywords ortners syndrome, bicuspid aortic valve, vagus nerve, schwannoma, hoarseness of voice. Pdf schwannomas arising from cervical vagus nerve is an extremely rare benign neoplasm. The mass was found at surgery to be in close proximity with the aortic arch and the left pulmonary hilum, alongside the left. This is a site geared to acoustic neuromas but it has been a life saver for myself and hundreds of others.
In the last 40 years, 30 cases at most have been reported, of which only 2 have been published in spanish. Vagal nerve schwannoma usually occurs between the third and fifth decades of life, it does not show sex predilection both sexes being equally affected and it most often presents as a painless, slowgrowing, lateral neck mass. Endoscopic resection of cervical vagal nerve schwannoma xi yang, mm, junlan liu, mb, linjun fan, phd breast disease center, southwest hospital of the third military medical university, chongqing china all authors. The gold standard for cervical vagal schwannomas is their complete surgical excision being often technically challenging in order to preserve the neural pathway of the vagus nerve and the. After surgery, the patient was resolved completely of his symptomatology, and he retained his nerve function. Cervical vagal schwannoma is an uncommon, benign neoplasm. A case report demah alayadhi1, abdullah arafat 2, mohammed elkarim2, mohammed alessa2, ali alassiri3 1 college of medicine, king saud ben abdulaziz university of health sciences. The mass was found at surgery to be in close proximity with the aortic arch and the left pulmonary hilum, alongside. A schwannoma, also known as an acoustic neuroma, is a benign nerve sheath tumor composed of schwann cells, which normally produce the insulating myelin sheath covering the peripheral nerves. Schwannomas develop in patients over a wide age range 1887 years, with a median between 53 and 68 years. This illustration shows a schwannoma of the tibial nerve in the leg. In the cases treated with intracapsular enucleation, only one case 20% maintained normal postoperative neurological function of the five vagal schwannomas.
Complete surgical excision is the treatment of choice. Case report a suspected palatine tonsil tumor hides an. Diagnostic and therapeutic hallenge jiten n 1, puspakishore m 1, sudhiranjan th 1, upasana r 1, sobita p 1, kalpana th 2 1 regional institute of medical sciences, 2 manipur state health services, manipur, india astrat schwannomas arising from the cervical vagus nerve are extremely rare benign neurogenic. Schwannome sind seltene, meist gutartige nervenscheidentumoren. A schwannoma is a usually benign nerve sheath tumor composed of schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves. In our case, the schwannoma was seen arising from the vagus nerve and patient presented with neck mass without any other clinical features. Oct 22, 20 vagal nerve schwannoma clinically mimicking gastrointestinal stromal tumor. Although the tumor capsule was not dissected from the vagus nerve, hoarseness and dysphagia. The patient reported complete resolution of her severe dry cough after surgery. Imaging characteristics of schwannoma of the cervical. Among the parapharyngeal tumours, salivary gland tumours are the commonest, followed by schwannomas, which are slow growing benign tumours. Leaving the part that is still attached to your vagus nerve. Barium swallow showed narrowing of the distal esophagus. Ct and mr findings were nonspecific, suggest ing a primary glioma.
This article presents 2 additional cases of this rare entity with associated clinical and unique radiographic findings. Pdf a schwannoma, also known as an acoustic neuroma, is a benign nerve sheath tumor composed of schwann cells, which normally produce the insulating. We describe a rare case of giant schwan noma that most likely originated in the vagus nerve, with an incidence lower than 6%. Diagnosis of benign schwannoma should be made before excision of the lesion to avoid an unnecessary esophagectomy. S chwannomas are benign neoplasms arising from myelinating schwann cells. Thoracoscopic excision of a malignant schwan noma of the intrathoracic vagus nerve. The accepted treatment of schwannomas is surgical resection. Unnecessary investigation and procedure should be avoided in order to reduce morbidity as well as improves patients quality of life. Vagal schwannoma involving esophagus the annals of. Cervical vagal schwannoma presenting as paroxysmal cougha. Neoplasms of the vagus nerve include paragangliomas 50%, schwannomas 31%, neurofibromas 14%, and neurofibrosarcomas 6%. Ct and mr findings claudio di biasi, guido trasimeni, massimo lannilli, elisabetta polettini, and gianfranco gualdi summary. Case report parapharyngeal neck schwannomas with unusual.
Schwannoma, originating from the cervical vagus nerve, is an extremely rare neoplasm that usually occurs in men between the 3rd and 6th decades of life. Injury to the vagus nerve causes hoarseness and dysphasia after the operation. Extracranial cervical vagus nerve schwannomas are quite rare and we found in english literature only 3 cases. Surgical resection is the treatment of choice even if its close relationship with nerve fibres, from which it arises, threats vagal nerve preservation. The tumor was embedded in the wall of the esophagus.
Kissing neurinomas of the vestibular and vagal nerves patrick fransen md catherine dozier md nicolas detribolet md geneva university hospital, service of neurosurgery, geneva, switzerland we report the clinical and neuroimaging features of a patient with double schwannomas originating from the vagal and vestibular nerves. A rare complication of vagal schwannoma family physician. Vagal nerve schwannoma usually occurs between the third and fifth decades of life, it does not show sex. Cervical vagal schwannoma is an extremely rare entity, slow growing, solitary, asymptomatic benign neck mass associated with vagus. Cervical vagal schwannomas constitute about 25% of neurogenic. After biopsy and diagnosis of benign schwannoma, it was dissected from the esophageal muscle and. We hereby present two cases of parapharyngeal schwannomas, one which had presented as an intraoral mass and the other. Vagal nerve schwannoma a rare neoplasm with a rare presentation and newer surgical management technique.
Neurofibromatosis type 2 associated with cranial nerve. Schwannomas can arise from any peripheral nerve containing schwann cells, including cranial nerves. Anatomically, vagus nerve is situated within the carotid sheath posterior to the internal carotid artery. They most commonly originate from the v, vii, iv, x, xi and xii cranial nerves. As a result, vagal schwannomas typically displace in ternal carotid artery anteriorly and. Ultrasonography, computed tomography, and magnetic resonance imaging were performed to differentiate preoperatively between schwannomas of the vagus nerve and schwannomas of the cervical sympathetic chain by observing the position of schwannomas in regard to the surrounding blood vessels. The majority of patients present with insidiously growing.
We report a case of vagal schwannoma correctly identi. Extracranial schwannomas are most often found in the parapharyngeal space, commonly involving the vagus nerve to cervical sympathetic trunk. Pdf large pure intracranial vagal schwannoma roberto. Imaging of carotid canal sympathetic plexus schwannoma j. Report of four cases med j malaysia vol 67 no 3 june 2012 347 there is general agreement concerning the value of magnetic resonance imaging in the preoperative workup as it is.
Half of the parapharyngeal schwannomas originate from the vagus. A rare case of vagal schwannoma neurilemoma with involvement of the distal esophagus is presented. The rostral ventrolateral medulla is thought to serve as a final common pathway for the integration of central cardiovascular information and to be important for the mediation of central pressor responses. Imaging characteristics of schwannoma of the cervical sympathetic chain. Sonographic diagnosis of a cervical vagal schwannoma. A parapharyngeal space schwannoma arising from the vagus. Mri reveals a hyperaemic mass with signal flow voids on t2 figures 3, 9b sometimes giving a classic saltandpepper sign. May 17, 2019 benign tumors can occur in nerves, muscle and bone. Vagal paraganglioma presenting as a neck mass associated. Ct and mri evaluation of nerve sheath tumors of the cervical. Webmd explains the causes and treatment of nerve sheath tumors known as neurofibrosarcoma and schwannoma. A schwannoma is a type of nerve tumor of the nerve sheath. We established a strategy for functional preservation in extracranial head and neck schwannoma treatment by using an emg system during tumor resection. Nerve palsy is a common complication of the vagal schwan noma removal, but in many cases it recovers spontaneously when the neural integrity is preserved.
Vagal nerve schwannoma usually occurs between the third and fifth. Cervical vagal schwannomas with cystic degeneration changes are extremely rare. Chest radiography, ct scan and mri showed a well circumscribed mass, 5. Pdf vagal nerve schwannoma a rare neoplasm with a rare. Head and neck schwannomas comprise 2545% of all extracranial schwannomas. Apr 08, 2011 on ct images, vagal schwannomas appear as welldefined masses, usually of higher attenuation than muscle on contrastenhanced images. Among them, benign neurogenic tumors are the most common types, most of which are represented by vagus nerve schwannomas and. The sheath surrounding nerve cells outside the central nervous symptoms is made up of schwann cells.
Benign peripheral nerve sheath tumors are usually divided into 2 groups. Multiple schwannomas occurring in patients without neurofibromatosis nf are rare and have recently been referred to as schwannomatosis. Differentiation between schwannoma of the vagus nerve and schwannoma of the cervical sympathetic chain by. Preoperative diagnosis of vagal and sympathetic cervical. Vagus nerve benign tumours schwannoma magnetic resonance imaging riassunto. Benign tumors can occur in nerves, muscle and bone. Spinal schwannomas are benign nerve sheath tumors within the spinal canal, typically arising from spinal nerve roots and it is the most common nerve sheath tumor of spine 11. They are adherent to nerve of origin vagus nerve schwannomas are general benign growing at the rate of 2. Vagal paragangliomas are rare neuroendocrine tumors arising from the neural crestderived paraganglionic tissue surrounding the vagus nerve, typically presenting as a neck mass associated with hoarseness or pulsatile tinnitus. Horners syndrome may be seen in a schwan noma of the cervical sympathetic chain.
Neurilemomas also known as schwannomas or neuromas account for 55% of these tumors. Four cases of schwannoma originating from the lower cranial nerves are presented. They are usually rare, slowgrowing tumours and reported to occur in patients between 30 and 50 years of age. The most common type of benign schwannoma is the acoustic neuroma. Acute esophageal hemorrhage from a vagal neurilemoma. We emphasize that rare diseases can mimic other pathologies. Schwannoma originating from the vagus nerve within the mediastinum is a rare, usually benign tumor. For a discussion of schwannomas located at specific sites, please refer to the relevant articles listed below. A ct with contrast showing a left carotid body tumor. Schwannomas of head and neck regioncommonly arise from the vestibular and vagus nerve. Vagus nerve schwannoma is a benign neoplasm that usually presents as an asymptomatic slow growing mass, and its.
Jugular foramen syndrome, or vernets syndrome, is characterized by paresis of the glossopharyngeal, vagal, and accessory with or without the hypoglossal nerves. Schwannoma of the vagus nerve, a rare middle mediastinal neurogenic tumor. There is the partial removal where they take most of the tumour out. Cervical vagal schwannoma is a benign, slowgrowing mass, often asymptomatic, with a very low lifetime risk of malignant transformation in. Pdf schwannoma originating from the cervical vagus nerve is an extremely rare neoplasm. Cervical vagal schwannoma is a rare entity among lesions presenting as a neck mass. A few months after excision ofthe primary tumor, multiple metastases appearedin lung, cervical spine, and neck lymph nodes. Diagnosis and management of extracranial head and neck. A schwannoma can affect any of the cranial nerves with the exception of the olfactory and optic nerves as these do not contain a schwann cell sheath. Rare presentation of cervical vagal schwannoma in adolescence. Schwannoma of the vagus nerve, a rare middle mediastinal. Variousimaging featureshadbeendescribedtodi erentiatethem,whichcon. Imaging of carotid canal sympathetic plexus clinical report. Schwannomas are homogeneous tumors, consisting only of schwann cells.
The tumorprobablyarose in the vagal nerve, and the trachea was involved. Endoscopic resection of cervical vagal nerve schwannoma. Vagal schwannoma to the the annals of thoracic surgery. The clinical features, diagnosis, management and pathological fi ndings of cervical vagal schwannoma are discussed. Vagal schwannomas present several unique clinical and therapeutic challenges. Schwannomas of cervical sympathetic nerve displace both carotid and jugular vessels, without separating them, whereas carotid arteries are usually separated from the internal jugular vein in vagus nerve schwannomas. Brachial plexus schwannoma constitute only about 5% of schwannomas. Case report a 43yearold woman presented to our institution. A rare case of vagus nerve schwannoma presenting as a neck. Amongthese,vagalschwannomasaremostcommonfollowedbycarotidbodytumors. Surgical treatment is generally pursued, taking care to avoid nerve damage 2, 3. In this article, we provide background information regarding surgical approaches to vestibular schwannomas and discuss and illustrate these approaches in terms of imaging and surgical techniques, anatomic variants, and complications early complications, postoperative hearing loss, delayed complications, tumor recurrence. Complete surgical excision is the treatment for vagus nerve schwannoma. Ct with contrast showing a poststyloid vagal schwan noma displacing the internal carotid artery anteromedially figs 4a and b.
A case of intracerebral schwannoma in a 19yearold man is reported. Carotid body tumors, cervical sympathetic chain schwan nomas, and vagal schwannomas have common location in neck that is the retrostyloid compartment of parapharyngeal space. We provide an extensive revision of all reported cases we found in literature. These tumours develop from a type of cell called a schwann cell, which gives them their name. Vagal nerve schwannoma usually occurs between the third and. Journal of cardiothoracic surgery schwannoma of the vagus nerve, a rare middle mediastinal neurogenic tumor. The esophagus and small intestine are rarely affected. Cervical vagal nerve schwannoma is an extremely rare, and slow. A case of a cervical vagal schwannoma, in a 33year old male with a previous medical history of malignant lymphoma, is described. In this report we suggest the occurrence of cervical schwannoma in patients with nf2 by demonstrating 2 such cases involving cervical schwan noma derived from the vagal or hypoglossal cranial nerve. Approximately half of the reported parapharyngeal schwannomas arise from the vagus nerve. The patient was eventually discharged from the hospital without any complication. This article provides a general overview of schwannomas. Since the first report of a schwan noma of the cervical vagus in 1926 by sekiga chi and oijie, there have been trickling case reports and a few short surgical series on nerve sheath tumors of the cervical segment of the vagus nerve 2, whereas the cases described in the radiology literature are mostly.
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