Splaying of the carotid bifurcation caused by a cervical sympathetic chain schwannoma

Splaying of the carotid bifurcation revealed by an imaging study is usually indicative of a carotid body tumor, but there are other possibilities. To promote awareness of a cervical sympathetic chain schwannoma as another cause of splaying of the carotid bifurcation, we present a case of cervical sympathetic chain schwannoma, with an additional 7 cases in the English-language literature, and discuss the relationship between the great vessels of the neck and a carotid body tumor or a schwannoma of the cervical sympathetic chain or vagus nerve from an anatomic viewpoint. We conclude that splaying of the carotid bifurcation with hypervascularity suggests a carotid body tumor, whereas in cases without hypervascularity, a cervical sympathetic chain schwannoma is another possibility. Vagus nerve schwannomas can separate the internal jugular vein and internal carotid artery, but seldom widen the carotid bifurcation.     

Parapharyngeal schwannoma of the cervical sympathetic chain in a child

Parapharyngeal schwannomas of the cervical sympathetic chain are benign tumors that have not been reported in children, compared to parapharyngeal neuroblastomas. We present, to our knowledge, the youngest reported case of parapharyngeal schwannoma of the cervical sympathetic chain. The nerve of origin could be clearly ascertained when excision was made through a cervical approach.     

El síndrome del primer mordisco

First-bite syndrome consists of the appearance of pain in the parotid at the beginning of mastication, due to damage to the cervical sympathetic chain or the sympathetic plexus innervating the parotid gland. Clinical presentation in a patient who has undergone surgery of the parapharyngeal space suggests the diagnosis.We report here the case of a patient who presented first-bite syndrome after being operated on for a cervical sympathetic chain schwannoma.     

Cervical sympathetic chain schwannoma

OBJECTIVES: Schwannomas are benign, slow-growing tumors that arise from nerves. Those originating from the sympathetic cervical chain are rare. We describe our experience with the clinical presentation, surgical management, and outcomes of patients with this pathology. STUDY DESIGN: Retrospective chart review of a case series in a tertiary referral center. METHODS: Four cases of cervical sympathetic chain schwannomas were reviewed. Patients presented with either an asymptomatic neck mass discovered on routine physical examination (1 patient), an enlarging neck mass (2), or an acute onset of a Horner's syndrome (1). All patients underwent preoperative imaging (magnetic resonance imaging, computed tomography, or both). RESULTS: The location and soft-tissue characteristics of the mass, along with displacement of the carotid sheath vessels, were typical of a cervical sympathetic chain schwannoma. All patients underwent surgical excision of the mass. Postoperative Horner's syndrome was encountered in all patients. First bite syndrome was encountered in two patients. CONCLUSIONS: Cervical sympathetic chain schwannomas are rare tumors. Preoperative imaging characteristics facilitate the diagnosis. First bite syndrome can occur and may be debilitating postoperatively. Long-term prognosis is excellent.     

Schwannoma of the neck

Sixteen cases of schwannomata of the neck were included in this study. Clinical and pathological features were analysed. Pre-operative diagnosis was always difficult and was achieved in only three cases. The lesion should be suspected whenever examining the neck for a solitary swelling of long standing. Schwannomata of the cervical vagus or sympathetic chain usually bulge into the pharynx and present as parapharyngeal tumours. Neurological deficits were absolutely absent in our cases. Every attempt should be exerted to spare the involved nerve even on the expense of leaving behind a part of the tumour which is definitely benign and having practically no tendency for malignant change.     

Schwannoma of the cervical sympathetic chain

Two cases of schwannomas of the cervical sympathetic chain (CSC) are described. Eleven clearly documented cases have been described in the previous literature. These tumours are usually solitary, asymptomatic cervical masses that occur within the upper carotid sheath or parapharyngeal space. The lesions tend to be hypervascular, but not to the same extent as chemodectomas. Angiography may be employed for differential diagnosis. Schwannomas of the CSC are usually readily excised by a cervical approach. Horner's syndrome is a frequent post-operative sequel, despite anatomical preservation of the sympathetic chain. This neurological impairment is usually asymptomatic. Recurrences and malignant degeneration rarely occur.     

Synchrones Schwannom des N. vagus und des zervikalen sympathischen Grenzstrangs

Schwannomas are rare neural sheath tumors which are generally benign. Up to 45% of all schwannomas originate in the head and neck region. In the parapharyngeal space (PPS) they may arise from any of the lower cranial nerves IX, X, XI and XII or from the cervical sympathetic chain. We report a unique case of a synchronous schwannoma of the vagal nerve and the cervical sympathetic chain in a patient without neurofibromatosis.     

Cervical sympathetic schwannoma with postoperative first bite syndrome

Extracranial schwannomas occurring in the head and neck region may arise from cranial, peripheral or autonomic nerves. Determination of the nerve of origin is not often made until the time of surgery. Schwannomas arising from the cervical sympathetic chain are extremely rare. These interesting tumors along with schwannomas in general and the remaining class of neurogenic tumors are known for their ability to mimic the physical and radiological findings of carotid body tumors. Surgery is the treatment of choice and major complications are infrequent. However, we report a case of cervical sympathetic chain schwannoma with postoperative first bite syndrome.     

First-bite syndrome: a review of 29 patients with parapharyngeal space tumor

Objective

The aim of this study is to clarify the etiology of first-bite syndrome.

Methods

A total of 29 consecutive patients treated with a surgical resection for parapharyngeal space tumor between April 1999 and April 2005 were retrospectively reviewed.

Results

Nine cases of first-bite syndrome were developed. Eight of those patients had undergone surgical ablation of the cervical sympathetic chain and/or external carotid artery. In two patients who underwent ablation of the sympathetic chain but in whom first-bite syndrome did not develop, the superior cervical ganglion was probably preserved.

Conclusion

The present findings strongly indicate that loss of sympathetic innervation to the parotid gland from the superior cervical ganglion causes first-bite syndrome. They also suggest that some residual or autonomous activity of the superior cervical ganglion could prevent development of first-bite syndrome even if the lower sympathetic trunk was ablated.     

Differentiation Between Schwannoma of the Vagus Nerve and Schwannoma of the Cervical Sympathetic Chain by Imaging Diagnosis

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. Ultrasonography also permitted direct visualization of the vagus nerve, so its position relative to the schwannoma could be examined. In schwannomas of the vagus nerve the schwannoma grew between the common carotid artery and the internal jugular vein or between the internal carotid artery and the internal jugular vein, resulting in an increase in the distance between the artery and vein (separation). In schwannomas of the cervical sympathetic chain, no separation was observed between the internal jugular vein and the common carotid artery or internal carotid artery. Ultrasonography with a 7.5-MHz transducer showed the derivation of the tumor from the vagus nerve in schwannomas of the vagus nerve but showed the vagus nerve on the tumor surface in schwannomas of the cervical sympathetic chain.     

Primary neuroblastoma: a rare cause of a retropharyngeal mass in a neonate

Neuroblastoma is the most common extra-cranial solid malignancy in children and the most common tumour occurring during infancy. This tumour arises from undifferentiated precursor cells of the sympathetic nervous system. The abdomen (65 per cent) is the most common site for these tumours, followed by the throat (15 per cent), pelvis (5 per cent) and cervical region (5 per cent). We report a case of primary retropharyngeal neuroblastoma in a three-week-old baby boy presenting with upper airway obstruction.     

Malignant granular cell tumour of the cervical sympathetic nerve trunk

Granular cell tumour is a rare neoplasm that can occur in various sites. This report describes a 48-year-old female with a malignant granular cell tumour originating from the cervical sympathetic nerve trunk, who presented with hyperaesthesia of the left shoulder. The tumour had a maximum diameter of 86 mm and involved adjacent tissues directly. Histopathologically, it showed considerable variation in the size and shape of nuclei, with occasional mitosis and an abundant granular cytoplasm. Immunohistochemically, the tumour reacted positively for S-100 protein and neuron-specific enolase, indicating its neural origin. Following total local excision of the lesion, the patient has been well without recurrence.     

Parapharyngeal space schwannomas: preoperative imaging determination of the nerve of origin

OBJECTIVES: To determine if preoperative radiographic cross-sectional images can predict the nerve of origin of a parapharyngeal schwannoma and, specifically, whether it originates from the vagus nerve or the cervical sympathetic chain. DESIGN: A retrospective review. SETTING: Academic medical center. PATIENTS: The study population comprised 12 patients who underwent surgical resection of schwannomas of the parapharyngeal space. The nerve of origin was identified based on operative findings and postoperative physical examinations. Of the 12 patients, 11 underwent preoperative magnetic resonance imaging and 1 underwent preoperative contrast-enhanced computed tomography. A CAQ (Certificate of Added Qualification)-certified neuroradiologist reviewed the imaging studies, blinded to the surgically determined nerve of origin. For each case, it was predicted whether the tumor arose from the vagus nerve or sympathetic chain based on the location of the schwannoma with reference to the carotid sheath vessels. MAIN OUTCOME MEASURE: Identification of the nerves of origin using the displacement of vessels as a marker. RESULTS: At the time of operation, it was determined that 5 patients (42%) had schwannomas from the cervical sympathetic chain and 7 patients (58%) had schwannomas of the cervical vagus nerve. By imaging, the nerve of origin was successfully determined in 4 of 5 cases of sympathetic chain schwannoma (80%) and in 7 of 7 cases of vagal nerve schwannoma (100%). Schwannomas of the cervical sympathetic chain were found to displace both the carotid and jugular vessels without separating them. Vagal nerve schwannomas were found to separate the carotid arteries from the internal jugular vein. A vagal nerve schwannoma may also displace the sheath vessels posteriorly, without splaying them. CONCLUSIONS: Carotid and jugular vessel displacement, as determined by cross-sectional imaging, can predict the likely nerve of origin of a parapharyngeal space schwannoma. This determination allows for effective preoperative counseling regarding the expected sequelae of surgical resection.     

An unusual location and presentation of a cervical ganglioneuroblastoma

Separation of the internal carotid artery (ICA) and internal jugular vein (IJV) on computed tomography and magnetic resonance imaging is a radiographic feature that enables tumors of the cervical vagus nerve to be distinguished from nerve sheath tumors of the cervical sympathetic chain. The present case illustrates an exception. This 4-year-old boy presented with a left cervical mass, which demonstrated separation of the ICA and IJV and reversal of the position of the ICA and the external carotid artery (ECA). The patient underwent en bloc resection of the tumor, which arose from the superior sympathetic ganglion, and displaced the IJV laterally and the ICA medially. The pathological findings showed ganglioneuroblastoma, intermixed. This case demonstrates an unusual location and presentation of a cervical ganglioneuroblastoma.     

Dumbbell-shaped cervical spinal neurilemmoma presenting as neck mass

Cervical neurilemmoma may originate from any nerve sheath tissue in the neck including the vagus nerve, glossopharyngeal nerve, brachial plexus, sympathetic trunk and cervical spine. We report an unusual case of a dumbbell-shaped neurilemmoma arising from the cervical spinal roots in a patient who complained of having had a neck mass for several months. Computed tomographic scan and magnetic resonance imaging revealed a dumbbell-shaped tumour extending from the C4 spinal level through the intervertebral foramen into the right parapharyngeal space. Decompression surgery was performed first via the cervical approach. Five months later, the patient received laminectomy and a complete tumour excision. The symptoms and signs were significantly relieved without neurological sequelae. No evidence of recurrence was noted after one-year follow up. This two-staged operation could offer an alternative surgical approach yielding ideal therapeutic results in such a rare disease.     

Cervical sympathetic chain schwannoma masquerading as a carotid body tumour with a postoperative complication of first-bite syndrome

Carotid body tumours (CBT) are the most common tumours at the carotid bifurcation. Widening of the bifurcation is usually demonstrated on conventional angiography. This sign may also be produced by a schwannoma of the cervical sympathetic plexus. A 45-year-old patient presented with a neck mass. Investigations included contrast-enhanced CT, MRI and magnetic resonance arteriography with contrast enhancement. Radiologically, the mass was considered to be a CBT due to vascular enhancement and splaying of the internal and external carotid arteries. Intraoperatively, it was determined to be a cervical sympathetic chain schwannoma (CSCS). The patient had a postoperative complication of first-bite syndrome (FBS). Although rare, CSCS should be considered in the differential diagnosis for tumours at the carotid bifurcation. Damage to the sympathetic innervation to the parotid gland can result in severe postoperative pain characterised by FBS and should be considered in all patients undergoing surgery involving the parapharyngeal space.     

Bilateral nasal vascular responses to unilateral sympathetic stimulation

Unilateral preganglionic cervical sympathetic stimulation in the anaesthetized cat evoked vasoconstriction in both nasal cavities, dependent on stimulation frequency. Vasoconstriction in the contralateral cavity was 15--20% of that of the stimulated side. Similar findings were obtained on unilateral Vidian nerve stimulation. Vasoconstriction evoked in the sympathectomized nasal cavity by stimulating the opposite cervical chain was reduced but not abolished by sectioning the posterior nasal and ethmoidal nerves of the stimulated side. It is suggested that vasoconstrictor fibres reach the opposite cavity either in these nerves or by way of blood vessels, but more likely that sympathetic fibres from these pathways innervate blood vessels which supply both nasal cavities.     

Malignant schwannoma of the ENT area

Malignant schwannomas are uncommon in the head and neck. They are derived from Schwann cells of cranial, cervical or sympathetic nerves and occur as solitary tumours or in association with neurofibromatosis (von Recklinghausen's disease). We present a patient of 30 years of age with a tumour of the right nasal and paranasal sinus system and a 68-year-old woman with the first described intratracheal malignant epitheloid Schwannoma. After the diagnosis of a malignant Schwannoma wide surgical excision is the recommended treatment, depending on the individual case, eventually followed by radiation therapy. As the tumour is more aggressive in patients with neurofibromatosis, the prognosis is better for patients without neurofibromatosis.     

Neurofibroma of the cervical sympathetic chain presenting with Horner's syndrome

A case of neurofibroma arising from the cervical sympathetic chain and associated with ipsilateral Horner's syndrome in a patient with von Recklinghausen's disease has been presented. The clinical features and the pathology of the lesion have been discussed.     

Schwannoma of the cervical sympathetic chain. The Virginia experience

We present 4 cases of schwannomas arising from the cervical sympathetic chain. These lesions are uncommon and most often present as an asymptomatic solitary neck mass. Preoperative diagnosis can be difficult, even with the aid of computed tomography, magnetic resonance imaging, ultrasound, and angiography. While a paraganglioma can often be ruled out, exact determination of the nerve of origin is frequently elusive until the time of surgery. Operative excision remains the treatment of choice, often requiring sacrifice of a portion of the sympathetic chain. Postoperative Horner's syndrome is common, but does not appear to have an adverse effect on the patient.