Nerve Schwannoma Research Articles

Expression of ErbB-1 and ErbB-2 in meningioma

Meningiomas are benign intracranial tumours treated by neurosurgical resection. Residual or recurrent tumour may be treated with radiotherapy. Effective molecular therapy for residual and recurrent meningiomas is currently unavailable. We tested meningiomas for In-ErbB-1 and ErbB-2, and the levels were compared to normal brain, peripheral nerve, glioblastoma multiforme and vestibular schwannoma. Our findings with immunohistochemistry and western blot analysis indicate a level of ErbB-1 similar to normal peripheral nerve, but a high expression of ErbB-2 similar to the vestibular schwannoma and glioblastoma multiforme. These preliminary results implicate a potential role for ErbB-2 in the benign tumourigenesis of meningioma consistent with the theoretical hierarchical interactions of homodimers of ErbB-2 that may be associated with low signal transduction.

Wewnątrzbłędnikowe nerwiaki nerwu przedsionkowo-ślimakowego

Summary Aim Presenting and discussing the patient with intravestibular schwannoma of the vestibulocochlear nerve, the course of disease, audiologic and radiologic examination and treatment possibilities. Methods Analysis of clinical presentation and treatment of the patient with intravestibular schwannoma and review of available literature on intralabyrinthine schwannomas. Results A 34-year-old male was diagnosed with a tumor localized in the left vestibule and semicircular canals without accompanying vertigo at any time of the disease but with tinnitus and total deafness of the left ear. Preoperative diagnosis was established on the basis of MRI with contrast. Localization of the tumor laterally to the fundus of internal auditory canal was essential for the diagnosis. Surgical treatment was chosen via translabyrinthine approach. Postoperative course was uneventful. Discussion Intralabyrinthine schwannoma are rare pathology. Their occurrence implies that schwannomas can originate at any segment of vestibulocochlear nerve. Most commonly intralabyrinthine schwannomas are observed at intracochlear or inrtavestibular localization. Present trials of these tumors classification were presented. The symptoms emerging in the course of disease are hearing loss, tinnitus, and vertigo, thus there were described patients with intralabyrinthine schwannomas diagnosed and treated as Meniere disease. Treatment depends on the intensity of symptoms and tumor expansion, while approach is dependant on its localization. Conclusion Elaborate radiologic examination including MRI with contrast is essential in case of atypical course of disease with hearing loss, tinnitus and vertigo, or with early diagnosis of Meniere disease.

Schwannoma of the Vagus Nerve Masquerading as a Carotid Body Tumor

A 45-year-old male presented with increasing hoarseness and swelling of his right neck over 5 months. He was noted to have right vocal cord paralysis and hemiatrophy of his tongue. He was found to have a schwannoma of the vagus nerve with compression of the right hypoglossal nerve. One month after his surgery, the patient's tongue mobility was improving, he was not aspirating, his voice was better and his right vocal cord remained abducted. A discussion of this case is followed by a review of the literature surrounding this rare disease and this unique presentation.

Nonvestibular schwannomas: an evaluation of functional outcome after radiosurgical and microsurgical management

Nonvestibular cranial nerve schwannomas (NVCNS) are relatively rare tumors. We evaluated our experience with radiosurgical and microsurgical treatment alone and in combination for the management of NVCNS. The charts of 62 patients with NVCNS who were treated between 1993 and 2005 at our institution were reviewed. Patients diagnosed with neurofibromatosis type 2 were excluded. The patients underwent microsurgery and/or radiosurgery treatment. Trigeminal and jugular foramen schwannomas were the most common NVCNS tumors (n = 47), and the only two groups with sufficient numbers of patients to allow comparison of the three treatment approaches. In these two groups, the mean tumor volume was significantly higher in those who received combined therapy (8.59 +/- 2.29 cc), compared with radiosurgery (4.94 +/- 3.02 cc; p = 0.05) or microsurgery alone (5.38 +/- 3.23; p = 0.027). Patients who underwent radiosurgery alone were significantly older (67.7 +/- 13.3 years; p = 0.019) than those treated with microsurgery (55.3 +/- 13.7 years) or with both modalities (48.7 +/- 12.8 years). The Karnofsky Performance Scale scores were significantly higher (p < or = 0.05) at follow-up compared with baseline for all three treatment approaches. There was no significant change in the Glasgow Outcome scores before and after treatment. Microsurgery and radiosurgery can both be used to manage NVCNS tumors with excellent results. When treatment with either modality alone is not reasonable, tumors can be managed effectively with combined micro- and radiosurgery treatment.

Behandlungsstrategien eines Schwannoms des Nervus vagus am Beispiel des interdisziplinären Vorgehens bei einem ausgedehnten Tumor

INTRODUCTION: Schwannoma of the vagal nerve are rare and usually accidentally found by exploring a slowly growing and painless neck tumor. CASE: We report on a 49-year-old patient with an extensive schwannoma of the vagal nerve. The tumor grew from the brainstem to the lower neck vessel sheath and destroyed the skull base. The tumor had already involved all nerves of the foramen jugulare. The surgery was performed in cooperation with the neurosurgeons. Initially the cervical part of the tumor was removed by transcervical approach. Then the endocranial part of the tumor was removed by retrosigmoidal craniotomy and partial petrosectomy. DISCUSSION: We have observed 5 schwannomas of the vagal nerve in 47,000 patients for 16 years. The diagnostic and therapeutic procedure is determined by the preoperative nerve function. In case of an intact nerve and after verifying the diagnosis of a schwannoma a detailed information given to the patient is absolutely nessecarry before any intervention. If the nerve lost its function there are no contraindications to resect the tumor and the nerve. Tumors with an intracranial extension should be treated interdisciplinarily (ENT/neurosurgery).

Behandlungsstrategien eines Schwannoms des Nervus vagus am Beispiel des interdisziplinären Vorgehens bei einem ausgedehnten Tumor

Schwannoma of the vagal nerve are rare and usually accidentally found by exploring a slowly growing and painless neck tumor. We report on a 49-year-old patient with an extensive schwannoma of the vagal nerve. The tumor grew from the brainstem to the lower neck vessel sheath and destroyed the skull base. The tumor had already involved all nerves of the foramen jugulare. The surgery was performed in cooperation with the neurosurgeons. Initially the cervical part of the tumor was removed by transcervical approach. Then the endocranial part of the tumor was removed by retrosigmoidal craniotomy and partial petrosectomy. We have observed 5 schwannomas of the vagal nerve in 47,000 patients for 16 years. The diagnostic and therapeutic procedure is determined by the preoperative nerve function. In case of an intact nerve and after verifying the diagnosis of a schwannoma a detailed information given to the patient is absolutely necessary before any intervention. If the nerve lost its function there are no contraindications to resect the tumor and the nerve. Tumors with an intracranial extension should be treated interdisciplinarily (ENT/neurosurgery).

Sonographic diagnosis of a cervical vagal schwannoma

This case report illustrates the role of high-resolution sonography in the preoperative assessment of a schwannoma of the vagus nerve in the neck. Sonography identified the tumor in the right carotid space and determined its origin from the right vagus nerve, facilitating the surgeon's approach to preserve nerve function.

Vestibular Evoked Myogenic and Periocular Potentials after Vestibular Schwannoma Surgery

We analyzed the behavior of preoperative and postoperative vestibular evoked myogenic potentials (VEMPs) and vestibular evoked periocular potentials (VEPPs) in 3 patients who had vestibular schwannoma and underwent modified translabyrinthine surgery. We compared VEMPs and VEPPs, measured with both air-conducted (AC) and bone-conducted (BC) stimulations. Vestibular evoked potentials were measured both in the immediate postoperative period and some months later. At the immediate postoperative examination, VEPPs were preserved in all 3 patients with both AC and BC stimulations. The VEMPs showed stable or improved parameters in 2 of our patients and were absent in the third patient. At the follow-up examination, VEMPs did not show any significant change with respect to the previous evaluation. In contrast, VEPPs were absent in 2 of our patients with AC stimulation and in 1 patient with BC stimulation. Our results seem to indicate a greater stability and reproducibility of VEMPs compared with VEPPs. In 1 patient, who had a schwannoma of the inferior vestibular nerve, the preservation of VEPPs and the absence of VEMPs in the immediate postoperative period confirm that the saccule represents the origin of VEMPs. Furthermore, these results suggest that not only the saccule, but also the utriculus, could be implicated in the origin of VEPPs.

Distribution of Nonvestibular Cranial Nerve Schwannomas in Neurofibromatosis 2

To describe the prevalence and location of cranial nerve schwannomas, other than bilateral vestibular schwannoma, in patients with neurofibromatosis 2 (NF2). The NF2 Natural History Consortium prospectively gathered cranial magnetic resonance imaging for 83 patients across 3 annual evaluations. The time between the first and last evaluation was approximately 3 years. Forty-two patients (51%) had nonvestibular cranial nerve schwannomas (NVSs). Of these, 25 (60%) also had cranial meningiomas. Twenty-one of those without NVS (25% of 83) had at least 1 meningioma. The average size of the NVS was 0.4 cubic centimeters. Overall, there was no significant change in NVS size from Year 1 to Year 3 or from Year 1 to Year 2. The most common locations of the NVS were occulomotor and trigeminal. A family history of NF2 did not predict NVS location or growth. Nonvestibular cranial nerve schwannoma usually affect cranial nerves III and V, as was the case in our NF2 sample. Fortunately, neuropathies associated with these tumors are rare. In contrast, lower cranial nerve schwannomas, although also rare, are associated with swallowing difficulty, aspiration, and other sequelae.

Primary middle ear schwannoma

Schwannomas of the middle ear may originate from the nerves of the middle ear cavity or by extensions from neighboring structures. We present a case of a 51-year-old female patient with primary middle ear schwannoma believed to arise from Jacobson's nerve. The tumor was easily divided from the facial nerve and the chorda tympani nerve. Erosion of the promontory was noted, and the jugular foramen and posterior wall of the ear canal were preserved without destruction. The mass was successfully removed by a canal down mastoidectomy procedure, while preserving the hearing and facial nerve functions of the patient. To our knowledge, this is only the second reported case of a patient with Jacobson's nerve schwannoma.

Infratentorial Meningioma in an 8-Year-Old Child as First Sign of Neurofibromatosis Type 2

Meningiomas are rare intracranial tumors in pediatric patients. In contrast to meningiomas in adults, childhood ones have a poorer prognosis because of their high growth potential and tendency to recur. Meningiomas are often associated with neurofibromatosis type 2 (NF2) which is an autosomal-dominant disorder. In contrast to adults who primarily present with symptoms due to vestibular tumors, the initial symptoms in children with NF2 are subtle skin tumors, posterior capsular cataracts, or neurological signs secondary to cranial nerve(s) schwannoma excluding vestibular nerve, and/or brainstem or spinal cord compression. Here we report on the clinical, radiological, and histological findings in an 8-year-old boy who was diagnosed with an isolated infratentorial meningioma and a novel splice site mutation in the NF2 gene. The same mutation was detected in the boy's mother who suffered from hearing loss and tinnitus due to a bilateral vestibular schwannoma. Our patient demonstrates the need for molecular testing for NF2 gene mutations even in isolated childhood meningiomas although they do not fulfill the clinical criteria of NF2.

顔面神経麻痺で発症した顔面神経海綿状血管腫症例

We encountered a rare case of facial nerve paralysis caused by cavernous hemangioma of the facial nerve.The patient was a 47 year-old man with a chief complaint of food leaking from the left angle of the mouth starting in January 2001. He was diagnosed as having Bell's paralysis, and he was treated with stel-late ganglion block (SGB). However, paralysis did not improve. Furthermore, MRI demonstrated a tumor in the left temporal bone. He was referred to our department on April 17, 2001.The facial score was 0/40 points by the Yanagihara method. There was no sign of cranial nerves other than the left facial nerve. CT showed a soft tissue shadow in the attic.Facial nerve schwannoma was suspected, and tympanoplasty was performed on October 16, 2001. The tumor bled easily. A solid tumor about 6mm in diameter was removed en bloc. Pathological diagnosis was a cavernous hemangioma of facial nerve origin.When we diagnose facial palsy, paralysis due to a tumor should be listed in the differential diagnosis. Imaging is essential for the diagnosis.

Schwannome épithélioïde du nerf acoustique : une observation

Epithelioid schwannomas mainly affect peripheral nerves and are frequently malignant. They are rarely encountered intracranially. Only five cases involving the eight nerve have been reported in the literature. We report the sixth case of epithelioid schwannoma of the acoustic nerve diagnosed in a 57 year-old woman operated for neurinoma of the right cerebellopontine angle. The patient was readmitted for local recurrence nine months later. Histologically, the tumour was composed of round or polygonal atypic cells associated with spindle cell areas. In our case, the local recurrence, nuclear pleomorphism and especially the high percentage of positive cells with p53 (80%) and Ki67 (30%) antibodies support the aggressive nature of the lesion.

A Pediatric Case of Neurofibromatosis Type 2 Presenting as a Plexiform Schwannoma of the Intraparotid Facial Nerve. A review of the world literature on the presenting pathology.

A Pediatric Case of Neurofibromatosis Type 2 Presenting as a Plexiform Schwannoma of the Intraparotid Facial Nerve. A review of the world literature on the presenting pathology.

A giant solitary schwannoma of the arm mimicking cyst hydatid.

We present a 77-year-old female with a size of 9.5×9×7cm3 giant schwannoma of musculocutaneous nerve volarly located in midarm, which was misdiagnosed as cyst hydatid on ultrasonographic and magnetic resonance image examination. There was not any neurologic sequele after the operation. In case of the similarity of their clinical and radiological findings, schwannoma should be included in the differential diagnosis of hydatid cysts especially in endemic countries.

Malignant schwanomma of parotid gland

Malignant neoplasms of facial nerve presenting as parotid mass are rare. The incidence of Malignant Schwannoma (MS) in general population is 0.001 % while its incidence in patients with Von Reclinghausen's neurofibromatosis (NF-1) is 4%. A case is presented of a 40-year-old male patient presenting with swelling in right parotid region increasing in size since last 10 years. Computed topography scan examination showed presence of parotid tumour with erosion of temporal bone and intracranial extension. Pre-operative tru-cut biopsy showed histology of pleomorphic adenoma. Patient was subjected to craniofacial resection and final histology turned out to be malignant schwannoma of facial nerve in the parotid gland. Since it's a rare intraparotid tumour, role of histopathology and immonohistochemistry in diagnosing the tumour and review of literature is presented.

Facial nerve schwannoma in the inner auditory canal and geniculate ganglion

Schwannoma of the vestibular nerve is the most common tumor found in the inner auditory canal. If a schwannoma of the facial nerve is found unexpectedly during surgery, the original surgical plan may have to be changed. In our series, we describe the symptoms, diagnostic procedures and therapy of this infrequent, benign tumor. In particular, we want to analyse the influence of diagnostic techniques, such as magnetic resonance tomography and electrophysiology, on the diagnosis of these tumors. Between 1975 and 2001, we diagnosed 17 cases of facial nerve schwannoma in the inner auditory canal or the geniculate ganglion. All patients were treated surgically. In 16 cases, we used the enlarged middle fossa approach and in the remaining patient the translabyrinthine approach was used. In 11 cases, the facial nerve could be saved but in six the nerve had to be rebuilt with a transplant. During the first years of our study, palsy of the facial nerve was a frequent finding. In the later years, a hearing impairment and tinnitus became the most frequent symptoms. Advances in magnetic resonance imaging have, in many cases, facilitated the differentiation between schwannomas of the facial nerve and the vestibular nerve over the last years. Signal enhancement at the geniculate ganglion is important for the diagnosis of facial nerve schwannoma. In individual cases, we found significant findings using electrophysiological procedures. In three cases, the temporal delay was elongated. Based on these findings, the therapeutic procedures can be discussed together with the patients.

Intraspinal schwannoma of the accessory nerve

The second example of a schwannoma originating from the cervical portion of the accessory nerve is reported.The tumour was diagnosed by MRI and confirmed by surgery. The tumour was small and was located entirely within the cervical subarachnoid space without causing any detectable neurological deficit.

Schwannoma of the spinal accessory nerve

The authors report a case of a schwannoma originating from the spinal root of the accessory nerve. The patient was a 40-year-old woman with chronic headache and blurred vision. A neurological examination failed to reveal any abnormal findings except for papilloedema. A computed tomographic (CT) scan showed hydrocephalus and a magnetic resonance image (MRI) on admission showed an enhancing extramedullary tumour located at the level of foramen magnum. The tumour was successfully removed and was verified to originate from the spinal root of the right accessory nerve. The hydrocephalus improved soon after surgery. The patient left hospital in a satisfactory condition. There are 15 cases of accessory nerve schwannomas reported in the literature; the authors report the 16th. Although accessory nerve schwannoma is very rare, one should consider it as one of the differential diagnoses for tumours at the foramen magnum.

Head and neck case of the day. Schwannoma of the right facial nerve.

Head and neck case of the day. Schwannoma of the right facial nerve.