Neurogenic tumors of the cervical vagus nerve: report of four cases and review of the literature

OBJECTIVE AND IMPORTANCE: Nerve sheath tumors arising from the cervical vagus nerve are extremely rare. These tumors most often present as asymptomatic, slowly enlarging, lateral neck masses and therefore often come initially to the attention of otolaryngologists and general surgeons. Because they are nerve tumors, however, neurosurgeons must be able to recognize and treat these rare entities. We report three cases of schwannoma and one case of neurofibroma of the cervical vagus nerve that were encountered at our center (Louisiana State University Medical Center) during a 31-year period. CLINICAL PRESENTATION: The patients ranged from 31 to 61 years of age at the time of presentation to Louisiana State University Medical Center. Presenting complaints included hoarseness, Horner's syndrome, and palpation of an enlarging, asymptomatic, cervical mass. Reviews of systems revealed episodes of aspiration for one patient and frequent respiratory illnesses for two patients. These episodes were possibly related to their tumors. Imaging studies demonstrated well-circumscribed masses in the region of the carotid sheath. INTERVENTION: Using microsurgical techniques, gross total resection of all four tumors was accomplished. For one patient, the vagus nerve needed to be divided and an end-to-end anastomosis was performed. For the other three patients, resection of the tumor was achieved with the vagus nerve in continuity. CONCLUSION: Vagal nerve schwannomas and neurofibromas in the neck are rare neoplasms. We present four cases of these benign tumors. The pathological features, epidemiological characteristics, presentation, differential diagnosis, and management are discussed. Gross total resection with preservation of the vagus nerve remains the treatment of choice.     

Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review

BACKGROUND: This study analyzes the management and outcomes of a series of 10 malignant peripheral nerve sheath tumors (MPNST) of the head and neck. METHODS: From 1984 to 2004, 10 patients underwent surgical treatment of a MPNST. We retrospectively reviewed presenting symptoms, radiological findings, surgical management, and follow-up status and performed a literature review. RESULTS: Eight tumors were located at the lateral skull base; 2 involved the vagus nerve in isolation. Two lesions were growing within the sinonasal tract. The most common presenting symptom was a rapidly enlarging cervical mass. Seventy percent of the tumors could be resected completely. Long-term follow-up showed a 2-year disease-specific survival rate of 50% and 5-year survival rate of 20%. Negative prognostic indicators were advanced tumor stage, early recurrence, and presumably also the presence of von Recklinghausen's disease. Postoperative adjuvant radiotherapy was found to make no difference in outcome. CONCLUSIONS: Although rare, MPNST is one of the most aggressive tumors in the head and neck area. Complete tumor removal is the mainstay of treatment and most important prognostic factor of MPNST. Adjuvant radiotherapy should be used to assist surgical excision in local control. The role of adjuvant chemotherapy remains controversial.     

A case of malignant peripheral nerve sheath tumor of the kidney

We report a case of malignant peripheral nerve sheath tumor (MPNST) of the kidney. A 50-year-old man was referred to our hospital because of left back pain. Computed tomographic scan and magnetic resonance imaging revealed a huge left retroperitoneal tumor (30 x 13 x 11 cm), which consisted of a solid tumor with calcification surrounding the cystic component. He underwent resection of the retroperitoneal tumor and additional descending colectomy. Histopathological examination revealed MPNST arising from severely atrophic renal parenchyma of giant hydronephrosis. He died of tumor metastasis to the lungs 3 months after the operation. To our knowledge, this is the fourth case of MPNST of the kidney, moreover the third case arising from the renal parenchyma to be reported in Japan.     

Cervical vagus nerve schwannoma

Schwannomas arising from cervical cranial nerves rarely present as neck swelling. Ultrasonography, computerised tomography, magnetic resonance imaging and fine needle aspiration cytology are helpful in reaching a diagnosis. Surgical excision is the treatment of choice. A case of large cervical vagus nerve schwannoma occurring in a young lady is presented with its management.     

Malignant peripheral nerve sheath tumor with Horner's syndrome: a case report

We report on a 42-year-old woman with malignant peripheral nerve sheath tumor (MPNST) arising from the cervical sympathetic nerve. A collar incision and partial sternotomy were performed at the second intercostal space. The mass was spindle shaped and connected to the sympathetic trunk on the cranial and caudal sides, and it compressed the left carotid sheath on the median side. After the patient's uneventful recovery from surgery, adjuvant radiotherapy was administered to the area of resection. The patient remains well 5 years after surgery with no evidence of recurrence.     

Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.

A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.     

Malignant Peripheral Nerve Sheath Tumor: An Unusual Cause of Intussusception

Malignant peripheral nerve sheath tumors (MPNST) are defined as any tumor arising from a peripheral nerve or showing nerve sheath differentiation. The majority of these tumors arise on the trunk, extremities, or head and neck region. The literature to date has fewer than 14 cases of MPNST arising in the gastrointestinal tract, and only two cases were ever reported in the small intestine, one of which was a recurrent disease. In this paper, we report the first US case of an MPNST arising in the small intestine and presenting as intussusception.     

A rare case of a solitary peripheral nerve sheath sarcoma of the scalp

Summary Malignant peripheral nerve sheath tumors are rare and most often appear in the clinical setting of von Recklinghausen's neruofibromatosis. On rare occasions they are seen in patients lacking any of the overt stigmata of this systemic disease. We report on a case of a peripheral nerve sheath sarcoma (PNSS) on the scalp and the back of the neck of a 13-year-old female arising in a solitary benign neurofibroma with no evidence of systemic neurofibromatosis. The tumor was examined by ultrastructural and immunohistochemical techniques as well as by routine histopathology. It was defined as a peripheral nerve sheath sarcoma, fibrosarcomatous type, of poorly differentiated perineurial cell origin. The possible significance of histogenetic subclassification in terms of surgical management and the oncological approach as well as the prognosis is discussed.     

Malignant peripheral nerve sheath tumour of the cervical vagus nerve in a neurofibromatosis type 1 patient.

One serious complication of neurofibromatosis type 1 (NF1) is the development of malignant peripheral nerve sheath tumours (MPNSTs). These malignancies often develop within pre-existing plexiform neurofibromas and their development is now thought to be associated with both tumour suppressor gene mutations and dysregulated growth factor signalling. Recent work demonstrates that the lifetime risk of malignant transformation is significantly greater than previously thought. Ionising radiation, a long-standing disease, particularly the presence of a large number of plexiform neurofibromas from an early age, are suggested risk factors. We present an NF1 patient who developed an MPNST of the cervical vagus nerve which was successfully treated with surgery. Close monitoring of patients with NF and a high level of suspicion towards rapidly enlarging and painful swellings is merited as these features may signify malignant transformation. Whether a positive history of MPNST in other affected family members predisposes the individual to a higher risk of malignant transformation is unclear.     

Cervico-mediastinal schwannoma of the vagus nerve: resection with intraoperative nerve monitoring

Schwannomas are usually benign, single, encapsulated, slow-growing tumours originating from cranial or spinal nerve sheaths. The vagus nerve involvement at the mediastinal inlet is very uncommon. For anatomical reasons, the resection of cervical and mediastinal schwannoma of the vagus nerve has a high risk of vocal fold paralysis. We describe the case of a 67-year-old female with a cervico-mediastinal schwannoma of the vagus nerve that we removed using the intraoperative neuromonitoring technique. The patient presented with mild hoarseness and complained of discomfort behind the jugular notch. Neck and chest computerized tomography described a 35 × 30 mm solid lesion behind the left clavi-sternal junction; preoperative fine needle aspiration cytology revealed schwannoma. Resection of the mass was performed with a cervical approach and the vagus nerve tumour was completely removed under continuous neuromonitoring (NIM-3^® System), preserving the vagus and the recurrent laryngeal nerve function. Pathology on the resected mass documented A-type schwannoma with “ancient schwannoma” areas. The intraoperative neurostimulation and neuromonitoring approach for the resection of vagus schwannoma are recommended because it may reduce the risk of injury to the vagus and to the recurrent laryngeal nerve.     

Retroperitoneal malignant peripheral nerve sheath tumor (MPNST) in a patient with neurofibromatosis I: a case report

A 64-year-old man who was diagnosed with type I neurofibromatosis was referred to our hospital with a pelvic mass incidentally found by ultrasonography. When examined by CT and MRI, the mass in the pelvis was 5 x 3 x 2 cm. In addition, another mass was found close to the obturator nerve at the S1-2 level. Transrectal biopsy was carried out, and a diagnosis of neurofibroma suspicious for low grade malignancy was made. We performed surgical resection of both of the tumors. The resected tumor was pathologically diagnosed as retroperitoneal malignant peripheral nerve sheath tumor (MPNST). We report our case and review the literature of MPNST detected in type I neurofibromatosis.     

Intrathoracic vagus nerve neurofibroma and sudden death in a patient with neurofibromatosis.

A 21 year old man with type 1 neurofibromatosis was found dead in the middle of the night. Postmortem examination revealed a large neurofibroma arising from the right intrathoracic vagus nerve, which might have contributed to his sudden death.     

Neurilemmomas of the cervical vagus nerve

Four patients were seen with neurilemmoma which arose in the cervical vagus nerve. Three tumors arose in the upper third of the lateral neck, and the remaining one in the lower third. Lateral neck mass was a prominent symptom in 3 patients, and parapharyngeal mass in the remaining one. The parapharyngeal mass bulged into the oropharynx in the tonsillar and retrotonsillar region and caused mild swallowig difficulty. Complete surgical extirpation by means of intracapsular enucleation was possible in all 4 patients. All patients remained free of disease for periods ranging from 15 to 75 months following surgery. It would appear that an intracapsular enucleation may be advisable for clearance of this kind of tumor.     

Sympathetic paraganglioma as an unusual cause of Horner's syndrome

BACKGROUND: Paragangliomas are rare tumors arising from paraganglionic tissue of neural crest origin. They are present in any location where autonomic ganglia are found. The most common location in the head and neck is the carotid body, followed by the jugular bulb and vagus nerve. METHODS: A 30-year-old woman with a slowly growing left neck mass, aniscoria, and left eyelid ptosis was found to have a vascular tumor consistent with a paraganglioma arising near the left carotid bifurcation. After preoperative embolization, the patient underwent resection of the tumor. RESULTS: The tumor was found to be arising from the left sympathetic trunk and did not involve any other surrounding structures. Histopathologic analysis revealed the typical findings of a paraganglioma. CONCLUSIONS: Sympathetic paragangliomas are exceedingly rare tumors in the head and neck and should be considered in the differential diagnosis when clinical and radiographic evidence suggest a paraganglioma. The presentation is typically a slow-growing neck mass with the presence of an ipsilateral Horner's syndrome.     

Surgical treatment of a left ventricular neurofibroma

Primary cardiac neurofibroma is a rare occurrence. We describe a case of left ventricular neurofibroma in a 56-year-old woman with Von Recklinghausen disease. Resection of the tumor with concomitant mitral valve replacement yielded a satisfactory clinical result, and histological examination of the resected tissue confirmed benign neurofibroma. The anatomic distribution of the vagus nerve plexus, which penetrates the epicardium and myocardium and courses over the left ventricular subendocardial surface, provides a tissue source from which this neurogenic tumor may arise.     

A resected case of malignant peripheral nerve sheath tumor (MPNST) necessary to distinguish from Askin tumor

We have treated a patient with malignant peripheral nerve sheath tumor (MPNST) for 7 years by interdisciplinary therapy. The diagnosis and treatment are reported with the clinical course so far. A 20-year-old man underwent surgical resection of a tumor on the left side of the chest in 1990, and a diagnosis of Askin tumor was made. In 1993, the disease recurred in the left axilla and was surgically resected. The surgical specimen was investigated histologically, and a diagnosis of MPNST was made instead of Askin tumor. Recently, a tumor began to grow in the left pleural cavity and was surgical resected. The tumor tissue contained both cells with oval and long spindle nuclei, which were intermingled with each other. Immunohistochemically, the tumor cells were positive for vimentin and some of them were positive for S100 protein. Because electron microscopy showed cytoplasmic processes on the tumor cell and basal lamina-like structures, a diagnosis of recurrent MPNST was made. Because this lesion resembled Askin tumor in its clinical manifestations and histological characteristics, differential diagnosis from Askin tumor was necessary. When a malignant soft tissue tumor arising from the mediastinum or chest walls is detected, immunohistochemical and electron microscopic studies should be performed. The diagnosis should be based on the overall findings including the location and clinical data.     

Sporadic malignant nerve sheath tumour of the oculomotor nerve

Malignant peripheral nerve sheath tumours (MPNST) are exceedingly rare in an intracranial location. In this report clinical and pathological evidence for the diagnosis of a MPNST arising from of the oclumotor nerve is presented. To our knowledge this is the first such case reported in the medical literature.     

Solitary neurofibroma arising from the infratemporal fossa in a child

Neurofibromas are derived from the nerve sheath and are commonly located in the head and neck region. They usually occur between the ages of 30 and 50 years. Neurofibromas arising from the infratemporal fossa are quite rare, especially in children. We describe a solitary neurofibroma arising from the infratemporal fossa in an 8-year-old boy who presented with a painless mass in his right cheek. Computed tomographic scan showed a soft-density, not well-circumscribed mass located in the right infratemporal fossa. The tumor was resected via the transmandibular approach with an excellent outcome. The histologic examination with immunohistochemical staining yielded the diagnosis of neurofibroma.     

C2 root nerve sheath tumors management

Background

Upper cervical nerve sheath tumors (NST) arising mainly from C2 root and to lesser extent from C1 root are not uncommon, they constitute approximately 5-12% of spinal nerve sheath tumors and 18-30% of all cervical nerve sheath tumors, unique in presentation and their relationship to neighbouring structures owing to the discrete anatomy at the upper cervical-craniovertebral region, and have atendency for growth reaching large-sized tumors before manifesting clinically due to the capacious spinal canal at this region; accordingly the surgical approaches to such tumors are modified. The aim of this paper is to discuss the surgical strategies for upper cervical nerve sheath tumors.

Methods

Eleven patients (8 male and 3 females), age range 28–63 years, with C2 root nerve sheath tumors were operated upon based on their anatomical relations to the spinal cord. The magnetic resonance imaging findings were utilized to determine the surgical approach. The tumors had extra- and intradural components in 10 patients, while in one the tumor was purely intradural. The operative approaches included varied from extreme lateral transcondylar approach(n?=?1) to laminectomy, whether complete(n?=?3) a or hemilaminectomy(n?=?7), with partial facetectomy(n?=?7), and with suboccipital craniectomy(n?=?2).

Results

The clinical picture ranged from spasticity (n?=?8, 72,72 %), tingling and numbness below neck (n?=?6, 54,54 %), weakness (n?=?6, 54,54 %), posterior column involvement (n?=?4, 26,36 %), and neck pain (n?=?4, 36,36 %). The duration of symptoms ranged from 1 to 54 months, total excision was performed in 7 patients; while in 3 patients an extraspinal component, and in 1 patient a small intradural component, were left in situ. Eight patients showed improvement of myelopathy; 2 patients maintained their grades. One poor-grade patient was deteriorated.

Conclusion

The surgical approaches for the C2 root nerve sheath tumors should be tailored according to the relationship to the spinal cord, determined by magnetic resonance imaging.     

Malignant peripheral nerve sheath tumour of penis

Malignant peripheral nerve sheath tumour (MPNST) is a rare variety of soft tissue sarcoma that originates from Schwann cells or pluripotent cells of neural crest origin. They have historically been difficult tumours to diagnose and treat. Surgery is the mainstay of treatment with a goal to achieve negative margins. Despite aggressive surgery and adjuvant therapy, the prognosis of patients with MPNST remains poor. MPNST arising from penis is a very rare entity; thus, it presents a diagnostic and therapeutic challenge. We present a case of penile MPNST in a 38‐year‐old man in the absence of neurofibromatosis treated with surgery followed by post‐operative radiotherapy to a dose of 60 Gray in 30 fractions and adjuvant chemotherapy with ifosfamide and adriamycin.