Cases reported "Cranial Nerve Neoplasms"

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1/68. Malignant peripheral nerve sheath tumour of the spinal accessory nerve.

    A 50-year-old man presented with a left-sided neck mass. Clinical examination revealed a large fluctuant 7 cm x 7 cm mass in the left posterior triangle. magnetic resonance imaging (MRI) revealed an encapsulated soft tissue lesion. He underwent exploration of the neck and a 14 cm by 8 cm mass enfolding the accessory nerve was identified and completely excised. Histological examination of the surgical specimen showed features in keeping with a malignant peripheral nerve sheath tumour (MPNST). We present the clinical and pathological features of this condition.
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2/68. Leptomeningeal metastases from ethmoid sinus adenocarcinoma: clinico-radiological correlation.

    A patient with sinonasal adenocarcinoma is presented with leptomeningeal metastases affecting multiple cranial nerves and spinal nerve roots. head and neck cancer is known to be an extremely rare source for leptomeningeal metastatic spread. The cranial nerves, the spinal cord and roots and the cerebral hemispheres can be affected in case of leptomeningeal metastatic spread. Examination of the CSF is the hallmark of the diagnosis if leptomeningeal metastatic spread is suspected, but this case illustrates that the combination of specific clinical features on one hand and specific lesions on the Gd-enhanced T1-weighted MRI study on the other hand is reliable enough to make a presumed diagnosis if the CSF analysis remains negative. We suggest that in our patient direct leptomeningeal spread occurred through the cribriform plate to the CSF, followed by further spread in a gravity dependent way.
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3/68. 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.
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4/68. Vagal paraganglioma. Report of a case surgically treated and review of the literature.

    The authors report the observation of one case of vagal paraganglioma occurred in a young woman. The tumor manifested itself as a left sub-mandibular tumescence; the very first diagnostic approach was achieved through echography, which showed a mass behind the internal carotid artery and compressing the internal jugular vein. The following examinations, represented by neck CT, NMR, angiography and fine needle aspiration initially directed towards the suspicion of chemodectoma. Only at operation, the anatomical situation of the tumor, which encapsulated the vagus nerve and the subsequent results of the hystological examination revealed the correct diagnosis of vagal paraganglioma.
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5/68. Perineural spread of squamous cell carcinoma of the lip: the importance of follow-up and collaboration.

    Perineural spread (PNS) of mucosal squamous cell carcinoma of the head and neck region occurs with a reported frequency of 2% to more than 27%. patients previously diagnosed with and treated for head and neck cancer should be closely followed by both their physician and their dentist in order to facilitate the coordination of care. This case history demonstrates the results that can occur when a team approach to head and neck cancer is not followed, especially in a patient who is an infrequent and somewhat reluctant health care utilizer. Despite mandibular pain, the patient, who had a history of a carcinoma of the lower lip and had developed PNS, was not referred to a dentist. In patients with a previous history of squamous cell carcinoma, sensory and/or motor changes must be closely monitored when there is a suspicion of PNS. The outcome of this case supports improved collaborations between physicians and dentists in following head and neck cancer patients.
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6/68. Intracranial adenoid cystic carcinoma--a case report.

    Adenoid cystic carcinoma (ACC) is a relatively common malignant neoplasm which occurs in the head and neck region, particularly in the salivary glands. It is a slow growing, locally aggressive neoplasm with a unique feature of perineural spread. Intracranial extension can occur by direct extension, hematogenous metastases or by perineural spread. However, the occurrence of primary intracranial ACC with no evidence of a primary, is rare, with only ten cases reported in reviewed English literature until now. One such case is presented here with brief review of literature. This patient showed a good response to treatment with surgery followed by post-operative radiotherapy and has remained disease free, 32 months after the completion of treatment.
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7/68. Superior oblique palsy in a patient with a history of perineural spread from a periorbital squamous cell carcinoma.

    A 74-year-old man experienced vertical diplopia. Two years earlier, he was diagnosed with a squamous cell carcinoma of the periorbital frontal skin, with perineural spread involving the ophthalmic division of the right trigeminal nerve and the right facial nerve. The clinical findings were consistent with a right fourth cranial nerve palsy. Computerized tomography and magnetic resonance imaging demonstrated a discrete mass involving the belly of the right superior oblique muscle. An anterior orbitotomy and biopsy demonstrated a mass extending into the belly of the superior oblique muscle. histology revealed an infiltrating squamous cell carcinoma. The possibility of perineural, direct, or metastatic spread to the superior oblique muscle should be considered in a patient with a history of squamous cell carcinoma of the head and neck. The authors believe this case to be the first report of superior oblique underaction due to involvement of the muscle by squamous cell carcinoma, presumably because of perineural spread. diagnosis was made possible by neuroimaging and histopathology. There was good short-term resolution of the patient's diplopia after radiotherapy.
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8/68. Schwannoma of the parapharyngeal space.

    A schwannoma (or neurilemmoma) of the parapharyngeal space is a rare tumor in the head and neck region arising from the sheath of cranial nerves and/or the sympathetic chain. In the early stages of growth, there is usually a lack of symptoms because of the anatomical localization and slow growth of the tumor. The large dimension of the mass at presentation makes it difficult to perform a functional surgery, and in many cases the operation worsens the nerve status. The choice of surgical approach is of paramount importance in both preserving nerve function and achieving vascular control. The authors report a case and discuss the English literature.
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9/68. A multifocal neurinoma of the hypoglossal nerve with motor paralysis confirmed by electromyography.

    A rare case of neurinoma in a 72 year-old Japanese woman derived from the hypoglossal nerve is reported. The tumour was composed of three interconnected nodules occurring simultaneously in the left submandibular and sublingual regions. The lesion, which presented as a neck mass, caused a slight left-sided hemiparesis of the tongue with tongue deviation to the affected side not noticed by the patient. An electromyographic (EMG) study revealed decreased muscle activity on the left side of the tongue muscle, indicating dysfunction of the hypoglossal nerve. EMG was useful for diagnosis.
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10/68. Concomitant vagal neurofibroma and aplasia of the internal carotid artery in neurofibromatosis type 1.

    We report the case of a patient with neurofibromatosis type 1 who had both aplasia of an internal carotid artery (ICA) and a vagal neurofibroma. To our knowledge, this is the first report in the literature of the simultaneous presence of these two rare disorders in a single patient. We believe that this is also the first report of an absence of an ICA in a patient with neurofibromatosis type 1. The patient was a 19-year-old woman who complained of a slowly growing neck mass. The mass occupied the right parapharyngeal space and upper cervical region. The patient had no other masses on physical examination, but widespread cafe au lait spots were evident. This led us to suspect the presence of a vagal neurofibroma. The tumor was removed, and pathology confirmed the diagnosis. No intracranial aneurysms were detected on cerebral angiography.
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