These tumours develop from a type of cell called a schwann cell, which gives them their name. Imaging of carotid canal sympathetic plexus clinical report. Mri reveals a hyperaemic mass with signal flow voids on t2 figures 3, 9b sometimes giving a classic saltandpepper sign. Vagus nerve schwannoma is a benign neoplasm that usually presents as an asymptomatic slow growing mass, and its. We report a case of vagal schwannoma correctly identi.
Approximately half of the reported parapharyngeal schwannomas arise from the vagus nerve. Vagal nerve schwannoma a rare neoplasm with a rare presentation and newer surgical management technique. Among them, benign neurogenic tumors are the most common types, most of which are represented by vagus nerve schwannomas and. As a result, vagal schwannomas typically displace in ternal carotid artery anteriorly and. Half of the parapharyngeal schwannomas originate from the vagus. Schwannomas develop in patients over a wide age range 1887 years, with a median between 53 and 68 years. These tumors are typically benign and slow growing. The esophagus and small intestine are rarely affected. Complete surgical excision is the treatment for vagus nerve schwannoma. Pdf large pure intracranial vagal schwannoma roberto. 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. Head and neck schwannomas comprise 2545% of all extracranial schwannomas. Such a lesion may be considered as a caveat to the paradigm of. A cervical vagal schwannoma mimicking a parathyroid cyst.
Cervical vagal schwannoma is a benign, slowgrowing mass, often asymptomatic, with a very low lifetime risk of malignant transformation in. This article provides a general overview of schwannomas. Ct and mr findings were nonspecific, suggest ing a primary glioma. The tumor was embedded in the wall of the esophagus. We hereby present two cases of parapharyngeal schwannomas, one which had presented as an intraoral mass and the other. Surgical treatment is generally pursued, taking care to avoid nerve damage 2, 3. The accepted treatment of schwannomas is surgical resection.
The most common type of benign schwannoma is the acoustic neuroma. 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. Vagal nerve schwannoma usually occurs between the third and. S chwannomas are benign neoplasms arising from myelinating schwann cells. The eighth cranial nerve is the most susceptible to schwannomas. 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. 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.
Cervical vagal nerve schwannoma is an extremely rare, and slow. Pdf schwannoma in the head and neck is usually arising from parapharyngeal space, but masseteric schwannoma is very rare. We provide an extensive revision of all reported cases we found in literature. A schwannoma is a usually benign nerve sheath tumor composed of schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves. 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. 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. Vagal schwannoma is a rare paraganglioma of head and neck and the best treatment is still controversial. 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. It is usually asymptomatic and presents as a painless, palpable mass in the neck. 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. However, surgery is not always recommended because of the indolent nature of the tumor and the risk of postoperative neural deficits. Chest radiography, ct scan and mri showed a well circumscribed mass, 5. Schwannomas are benign nerve sheath neoplasms arising from peripheral or cranial nerves. This nerve is the only nerve that runs from the brain all the way down through the body.
Barium swallow showed narrowing of the distal esophagus. 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. We established a strategy for functional preservation in extracranial head and neck schwannoma treatment by using an emg system during tumor resection. Keywords ortners syndrome, bicuspid aortic valve, vagus nerve, schwannoma, hoarseness of voice. Chronic progressive dysphagia was the only complaint related to the schwannoma. Variousimaging featureshadbeendescribedtodi erentiatethem,whichcon. Cervical vagal schwannoma is an uncommon, benign neoplasm. Diagnostic approach to patients with tinnitus kenneth s. More serious causes, such as meniere disease or vestibular schwan noma, can be excluded during the evaluation. Schwannoma of the vagus nerve, a rare middle mediastinal neurogenic tumor. Single case report schwannomas of the cervical vagal nerve are rare neoplasms, usually occurring between the third and the sixth decade of life. Acute esophageal hemorrhage from a vagal neurilemoma.
Cervical vagal schwannoma presenting as paroxysmal cougha. In treating schwannoma patients, it is critical to determine the origin of the tumor to preserve nerve function. Schwannoma originating from the vagus nerve within the mediastinum is a rare, usually benign tumor. Schwannoma is a benign tumor that originates from the neural sheath of schwann cells. The mass was found at surgery to be in close proximity with the aortic arch and the left pulmonary hilum, alongside the left. 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. In our case, the schwannoma was seen arising from the vagus nerve and patient presented with neck mass without any other clinical features.
Imaging characteristics of schwannoma of the cervical. Benign posterior mediastinal schwannomamultiple diagnostic. A rare complication of vagal schwannoma family physician. Complete surgical excision is the treatment of choice. Oct 22, 20 vagal nerve schwannoma clinically mimicking gastrointestinal stromal tumor.
They may be intracranial only or have both intra and extracranial extension. Vagal schwannoma involving esophagus the annals of thoracic. Schwannomas can arise from any peripheral nerve containing schwann cells, including cranial nerves. In the last 40 years, 30 cases at most have been reported, of which only 2 have been published in spanish. Metastatic papillary carcinoma of the thyroid may also enhance with contrast. 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. After biopsy and diagnosis of benign schwannoma, it was dissected from the esophageal muscle and. The rate of nerve palsy at 6 months after complete tumor resection and intracapsular enucleation was 100 1111 and 31% 516, respectively. The mass was found at surgery to be in close proximity with the aortic arch and the left pulmonary hilum, alongside. Ct and mri evaluation of nerve sheath tumors of the cervical. Hypoglossal nerve schwannoma in the submandibular region. They are usually rare, slowgrowing tumours and reported to occur in patients between 30 and 50 years of age.
Case reports five cases of nf2 were seen at kansai medical university hospital between 1992 and 1996. Schwannomas are homogeneous tumors, consisting only of schwann cells. A few months after excision ofthe primary tumor, multiple metastases appearedin lung, cervical spine, and neck lymph nodes. 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. Jugular foramen syndrome, or vernets syndrome, is characterized by paresis of the glossopharyngeal, vagal, and accessory with or without the hypoglossal nerves. Case report a 43yearold woman presented to our institution. Extracranial schwannomas are most often found in the parapharyngeal space, commonly involving the vagus nerve to cervical sympathetic trunk. They are usually slowgrowing benign lesions closely associated with the vagus nerve. Functional nerve preservation in extracranial head and. Endoscopic resection of cervical vagal nerve schwannoma. The majority of patients present with insidiously growing. Vagal schwannomas present several unique clinical and therapeutic challenges. Pdf vagal nerve schwannoma a rare neoplasm with a rare.
Journal of cardiothoracic surgery schwannoma of the vagus nerve, a rare middle mediastinal neurogenic tumor. This is a site geared to acoustic neuromas but it has been a life saver for myself and hundreds of others. Pdf a schwannoma, also known as an acoustic neuroma, is a benign nerve sheath tumor composed of schwann cells, which normally produce the insulating. Schwannoma originating from the cervical vagus nerve is an extremely rare neoplasm. 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. Ct and mri evaluation of nerve sheath tumors of the cervical vagus nerve. 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. A rare case of vagus nerve schwannoma presenting as a neck. Schwannoma can occur anywhere in the gi tract but is most common in the stomach, followed by the colon and rectum. There is the partial removal where they take most of the tumour out. Vagal nerve schwannoma usually occurs between the third and fifth. Carotid body tumors, cervical sympathetic chain schwan nomas, and vagal schwannomas have common location in neck that is the retrostyloid compartment of parapharyngeal space. Imaging characteristics of schwannoma of the cervical sympathetic chain. 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. One case report of a schwannoma involving the sympathetic plexus in the carotid canal has been reported previously. Vagal schwannoma involving esophagus the annals of. Webmd explains the causes and treatment of nerve sheath tumors known as neurofibrosarcoma and schwannoma. Pdf schwannomas arising from cervical vagus nerve is an extremely rare benign neoplasm. Ct and mri evaluation of nerve sheath tumors of the. Cervical vagal schwannomas with cystic degeneration changes are extremely rare. 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. Vagal nerve schwannoma usually occurs between the third and fifth decades of life, it does not show sex. Vagal schwannoma to the the annals of thoracic surgery. Thoracoscopic excision of a malignant schwan noma of the intrathoracic vagus nerve. Rare presentation of cervical vagal schwannoma in adolescence. Cervical vagal schwannomas constitute about 25% of neurogenic.
This article presents 2 additional cases of this rare entity with associated clinical and unique radiographic findings. Anatomically, vagus nerve is situated within the carotid sheath posterior to the internal carotid artery. Unnecessary investigation and procedure should be avoided in order to reduce morbidity as well as improves patients quality of life. Diagnosis and management of extracranial head and neck. 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.
Compression of the rostral ventrolateral medulla by a. Extracranial cervical vagus nerve schwannomas are quite rare and we found in english literature only 3 cases. They dont demonstrate any sex predilection, and they often present as slowgrowing, palpable neck masses, left. Benign tumors can occur in nerves, muscle and bone. Benign peripheral nerve sheath tumors are usually divided into 2 groups. It was suggested that paroxysmal cough during fnac or palpation of the mass was diagnostic for the vagal nerve schwannoma. The tumorprobablyarose in the vagal nerve, and the trachea was involved. Ct and mr findings claudio di biasi, guido trasimeni, massimo lannilli, elisabetta polettini, and gianfranco gualdi summary. 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. Amongthese,vagalschwannomasaremostcommonfollowedbycarotidbodytumors. A rare case of vagal schwannoma neurilemoma with involvement of the distal esophagus is presented. Schwannomas, less commonly called neurinomas or neurilemmomas, are benign tumors of schwann cell origin and are the most common tumor of peripheral nerves, including cranial nerves. Case 1 is a schwan noma of the vagus nerve in the parapharyngeal space. Neurofibromatosis type 2 associated with cranial nerve.
Mayo clinic surgeons carefully remove your schwannoma while taking care to preserve nerve fascicles that arent affected by your tumor. 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. Although the tumor capsule was not dissected from the vagus nerve, hoarseness and dysphagia. This illustration shows a schwannoma of the tibial nerve in the leg. Neurilemomas also known as schwannomas or neuromas account for 55% of these tumors. A schwannoma is a type of nerve tumor of the nerve sheath. Vagus nerve benign tumours schwannoma magnetic resonance imaging riassunto. Nov 26, 2009 schwannoma originating from the vagus nerve within the mediastinum is a rare, usually benign tumor.
An association between essential hypertension and neurovascular compression of the rostral ventrolateral medulla has been reported. We describe a rare case of giant schwan noma that most likely originated in the vagus nerve, with an incidence lower than 6%. In the parapharyngeal space, schwannomas most commonly arise from the vagus nerve and cervical sympathetic chain. A case of mediastinal malignant epithelioid schwan noma mes is reported. Sonographic diagnosis of a cervical vagal schwannoma. Neoplasms of the vagus nerve include paragangliomas 50%, schwannomas 31%, neurofibromas 14%, and neurofibrosarcomas 6%. A case of intracerebral schwannoma in a 19yearold man is reported. Hypoglossal and accessory nerve preservation is also necessary to prevent postoperative dysphagia. 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.
Cervical vagal schwannoma is a rare entity among lesions presenting as a neck mass. A ct with contrast showing a left carotid body tumor. In some cases, the history and the age of patients can render diagnoses incorrect. Surgical resection is the treatment of choice even if its close relationship with nerve fibres, from which it arises, threats vagal nerve preservation.
A schwannoma shwonoma is a tumour of the tissue that covers nerves, called the nerve sheath. Leaving the part that is still attached to your vagus nerve. 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. Schwannome sind seltene, meist gutartige nervenscheidentumoren. Multiple schwannomas occurring in patients without neurofibromatosis nf are rare and have recently been referred to as schwannomatosis. They are adherent to nerve of origin vagus nerve schwannomas are general benign growing at the rate of 2. Pdf schwannoma originating from the cervical vagus nerve is an extremely rare neoplasm. In the cases treated with intracapsular enucleation, only one case 20% maintained normal postoperative neurological function of the five vagal schwannomas.
We emphasize that rare diseases can mimic other pathologies. The operation was performed by the transcervical approach. Ct with contrast showing a poststyloid vagal schwan noma displacing the internal carotid artery anteromedially figs 4a and b. The tumor cells always stay on the outside of the nerve, but the tumor itself may either push the nerve aside andor. Four cases of schwannoma originating from the lower cranial nerves are presented. They most commonly originate from the v, vii, iv, x, xi and xii cranial nerves. Schwannomas of head and neck regioncommonly arise from the vestibular and vagus nerve. The patient was eventually discharged from the hospital without any complication. We report the case of patient diagnosed with intrathoracic vagus nerve schwannoma. 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. Differentiation between schwannoma of the vagus nerve and schwannoma of the cervical sympathetic chain by. Case report a suspected palatine tonsil tumor hides an.
A parapharyngeal space schwannoma arising from the vagus. Preoperative diagnosis of vagal and sympathetic cervical. Vagal paraganglioma presenting as a neck mass associated. I ntr aopera tive image of the schwan noma a the stoma ch, bthe vagal. Horners syndrome may be seen in a schwan noma of the cervical sympathetic chain. They are one of the two most common intradural extramedullary spinal tu. 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. 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. Schwannoma of the vagus nerve, a rare middle mediastinal. The patient reported complete resolution of her severe dry cough after surgery. 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. A case of a cervical vagal schwannoma, in a 33year old male with a previous medical history of malignant lymphoma, is described. Differentiation between schwannoma of the vagus nerve and. 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.
Such type of association is unique and new to voluminous medical literature. After surgery, the patient was resolved completely of his symptomatology, and he retained his nerve function. The clinical features, diagnosis, management and pathological fi ndings of cervical vagal schwannoma are discussed. Diagnosis of benign schwannoma should be made before excision of the lesion to avoid an unnecessary esophagectomy. Injury to the vagus nerve causes hoarseness and dysphasia after the operation. Clinically, schwannomas present as asymptomatic, slowly enlarging lateral neck. Brachial plexus schwannoma constitute only about 5% of schwannomas. Vagal refers to the vagus nerve which is the 10th cranial nerve. Apr 08, 2011 on ct images, vagal schwannomas appear as welldefined masses, usually of higher attenuation than muscle on contrastenhanced images. Kissing neurinomas of the vestibular and vagal nerves.
May 17, 2019 benign tumors can occur in nerves, muscle and bone. For a discussion of schwannomas located at specific sites, please refer to the relevant articles listed below. Cervical vagal schwannoma is an extremely rare entity, slow growing, solitary, asymptomatic benign neck mass associated with vagus. Among the parapharyngeal tumours, salivary gland tumours are the commonest, followed by schwannomas, which are slow growing benign tumours. Imaging of carotid canal sympathetic plexus schwannoma j. Case report a cervical vagal schwannoma mimicking a. B internal and external carotid arteries left carotid body tumor splaying of the angiogram of a by. The sheath surrounding nerve cells outside the central nervous symptoms is made up of schwann cells. 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.