Cases reported "Neurofibroma"

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1/34. 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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2/34. Cervical chordoma with vertebral artery encasement mimicking neurofibroma: MRI findings.

    A case of cervical chordoma in a 36-year-old white man with hypoesthesia in the neck and right shoulder, neck pain, and restricted neck mobility is presented. Plain radiographs of the cervical spine showed radiolucency of the body of C2 on the right side and enlargement of the right intervertebral foramen at C2-C3 level. Tumor encasement of the vertebral artery was demonstrated by MR imaging and confirmed by conventional arteriography. This proved to be particularly important for preoperative assessment.
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3/34. 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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4/34. Intradermal spindle cell/pleomorphic lipoma of the vulva: case report and review of the literature.

    BACKGROUND: Spindle cell/pleomorphic lipoma (SC/PL) is a benign adipose tissue tumor that usually affects the subcutaneous tissues of shoulders, backs, and neck region of middle-aged male patients. Histologically, it is characterized by the presence of primitive CD34-positive spindle cells arranged in short fascicles, bizarre floret-like multinucleated giant cells, mature adipocytes, and a small number of lipoblasts. Recently, an intradermal subset has been described, which mainly affects female patients and presents a wider antomical distribution when compared to the classical variant of SC/PL. methods: We report a case of intradermal SC/PL affecting the labium majus of a 56-year-old female patient. RESULTS: The histological examination disclosed the typical histological features, however the lesion showed poorly demarcated and infiltrative borders, as well as involvement of dermal nerves. The immunohistochemical analysis according to streptovidin-biotin-peroxidase technique showed immunoreactivity for CD34 and vimentin in the spindle cells, as well as S100 protein and vimentin in the adipocytic cells. CONCLUSIONS: To the best of our knowledge, this is the first case of intradermal SC/PL affecting the vulvar region. Care must be taken not to misdiagnosis this rare tumor as well-differentiated liposarcoma, cellular angiofibroma, solitary fibrous tumor, and cutaneous neurofibroma.
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5/34. March 2002: 28-year-old woman with neck and back pain.

    Following a car accident a 28-year-old female, complained of a sharp pain of the anterior and posterior base of the neck on expiration and with exertion. Subsequently, she noticed a feeling of discomfort in her back when lifting her arm above her head. Imaging studies revealed a tumor mass involving the third intercostal nerve on the right side of T2. The differential diagnosis included neurofibroma and neurilemmoma. This was followed annually and five years later an increase in size warranted a transthoracic, transpleural removal en bloc of this lesion. At surgery, a 3 cm soft tissue tumor engulfed the third intercostal nerve and extended into the third intervertebral foramen where the proximal part of the nerve root was enlarged. The right third intercostal nerve was dissected and removed along with the tumor, after negative nerve stimulation. Histopathological examination showed multiple enlarged coalescent lymphoid follicles with an onion skin appearance of tight concentric layering of small, uniform mature lymphocytes at the periphery, arranged in a targetoid fashion with broad mantle zones and relatively small germinal centers. The germinal centers of variable size included hyalinized blood vessels. Lollipop follicles were seen. The interfollicular stroma showed numerous hyperplastic collagenized capillaries within an inflammatory background. However, the perinodal soft tissue was replaced by numerous inflammatory cells, primarily lymphocytes. The final diagnosis was Castleman's disease, hyaline vascular type. Castleman's disease can mimic various tumors and because Castleman's disease is a rare reactive entity, its diagnosis is generally overlooked by radiologists and clinicians. It is likely that this mass arose from one of the posterior intercostal lymph nodes, situated in the paravertebral region, however the capsule was not readily seen and the sinuses were not apparent. Almost all previous cases of Castleman's disease, hyaline vascular type were described in the anterior mediastinum. Hyaline vascular Castleman's disease usually does not invade and replace neighboring structures. This case is unique because of its location and the local invasion of adjacent structures.
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6/34. Diffuse neurofibroma of scalp.

    Diffuse neurofibroma is an uncommon but distinct variety of neurofibroma, usually affecting trunk, head and neck regions of adolescents and young adults. The clinical features, gross macroscopic and histopathological findings are enunciated and the criteria for instituting the preferred modality of treatment for such lesions has been reviewed, stressing upon the need to exclude the neurofibromatoses preoperatively.
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7/34. Diffuse neurofibroma on the lower back.

    Diffuse neurofibroma is an uncommon form of neurofibroma, which occurs principally in children and young adults. This tumor presents most often in the head and neck region. We have seen a young girl with a diffuse neurofibroma on the lower back that was successfully excised.
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8/34. Benign solitary nerve sheath tumors of the spinal accessory nerve in the posterior triangle of the neck. Report of two cases.

    A case of solitary schwannoma and one of solitary neurofibroma originating from the spinal accessory nerve in the posterior triangle of the neck are described. Location of such neoplasms in this region is exceptional. The authors emphasize the importance of accurately enucleating the mass; when it is impossible to preserve the continuity of the neural pathway, nerve repair should be considered.
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9/34. Extramedullary sarcoid lesion mimicking intraspinal tumor.

    BACKGROUND CONTEXT: Spinal sarcoidosis is rare. Most spinal sarcoid lesions are intramedullary, and only three cases of extramedullary sarcoid lesions have been reported. PURPOSE: To describe a case of an extramedullary sarcoid lesion in a patient that did not have systemic involvement and to review the literature of spinal sarcoidosis. STUDY DESIGN/SETTING: Case report and review of the literature. PATIENT SAMPLE: Case report. OUTCOME MEASURES: Report of resolution of symptoms. methods/DESCRIPTION:The patient was a 33-year-old woman who had neck pain and pain radiating to the right scapula area and down the right arm into her hand and wrist. She also complained of numbness, tingling, muscle spasms and tremors, and had difficulty with writing. magnetic resonance imaging showed a mass that was extramedullary in the right lateral aspect of the spinal canal at the level of C5 and extending into the right C5-6 neuroforamen. Admitting diagnosis of neurofibroma was made. The patient underwent C4, C5 and C6 laminoplasty and gross total resection of an intradural extramedullary tumor. The lesion encroached on the neuroforamen on the right side involving the C6 nerve root, was grossly adherent to some of the rootlets and looked like a Schwannoma. Gross total resection of the tumor was performed. Pathological examination of the specimen showed a noncaseating granulomatosis consistent with sarcoid. Postoperative testing did not reveal systemic involvement of sarcoidosis. The patient was treated with corticosteroids. RESULTS: The patient made a satisfactory recovery, returned to work full-time, and had no complaints of neurological symptoms. CONCLUSIONS: An extramedullary sarcoid lesion is rare. Unlike intramedullary sarcoid lesions, it can be totally removed. If no systemic sarcoidosis is present, the patient can have a satisfactory recovery.
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10/34. Angiosarcoma arising from malignant schwannoma in a patient with neurofibromatosis.

    A 20-year-old man with von Recklinghausen disease had a right neck mass. Pathologic examination at excision showed angiosarcoma arising in a malignant schwannoma of the cervical brachial plexus. Comparison with previous reports revealed that angiosarcoma is a rare, highly aggressive sarcomatous element in malignant schwannoma arising in patients with neurofibromatosis (NF).
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