Cases reported "Neurofibroma"

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1/77. The changing faces of a parotid mass.

    The development of a neurofibroma in the temporal and parotid regions of the facial nerve is rare. We report a case occurring in a 32-year-old male, where the initial presentation was a parotid mass. Cytologically this was initially thought to be a pleomorphic adenoma. However, the diagnosis was then changed to a schwannoma and finally a neurofibroma after complete excision of the lesion. The case illustrates how fine needle aspiration cytology under ultrasound guidance, and even histological examination of an incision biopsy can sometimes fail to give the correct diagnosis. In this case magnetic resonance imaging was used to help plan definitive surgery.
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ranking = 1
keywords = schwannoma
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2/77. 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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ranking = 2
keywords = schwannoma
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3/77. Cystic lumbar nerve sheath tumours: MRI features in five patients.

    Intraspinal cystic lumbar nerve sheath tumours constitute an uncommon subset of tumours with distinct clinico-biological behaviour. The MRI findings in five such cases are presented. Four of these were cystic schwannomas and one was a cystic neurofibroma. The pathology, MRI findings and the differential diagnosis of these rare tumours are analysed.
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ranking = 1
keywords = schwannoma
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4/77. Pediatric pigmented dermatofibrosarcoma protuberans (Bednar tumor): case report and review of the literature with emphasis on the differential diagnosis.

    dermatofibrosarcoma protuberans (DFSP) is a fibrous tumor of intermediate malignant potential that usually affects the trunk of young to middle-aged adults. On histological examination, it is characterized by a monomorphous population of spindle cells arranged in a storiform or cartwheel pattern. Bednar tumor (BT), formerly known as storiform pigmented neurofibroma, is currently considered the pigmented variant of DFSP due to the histological and cytogenetic similarities between these two lesions. There are very few reports on BT affecting pediatric patients. We describe a case of BT affecting the dorsal aspect of the left forearm of a 6-year-old-male patient and emphasize the diagnostic clues to distinguish this unusual cutaneous neoplasm from other pigmented lesions, including pigmented (melanotic) neurofibroma (PMN), psammomatous melanotic schwannoma (PMS), neurocristic cutaneous hamartoma (NCH), and desmoplastic malignant melanoma (DMM). We would like to stress that surgical pathologists and dermatopathologists need to be aware of the prototypical histological appearance of BT as there is the risk of misdiagnosing it either as pigmented tumors associated with neurocutaneous syndromes, such as PMN and PMS, or as a highly malignant melanocytic neoplasm (DMM).
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ranking = 1.0766982490999
keywords = schwannoma, melanocytic
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5/77. Intraparotid facial nerve neurofibromas.

    OBJECTIVES: To provide an awareness of intraparotid facial nerve neurofibroma as a cause of parotid masses and to describe their characteristics and management considerations. STUDY DESIGN: Case report with literature review. methods: The medical records of three patients with intraparotid facial nerve neurofibromas are reviewed, and data concerning the patient's presentations, treatment, and disease course are presented with a review of the world's literature on intraparotid facial nerve neurofibromas. CONCLUSIONS: Tumors arising from the extratemporal course of the facial nerve are quite rare. The tumors arise from schwann cells and include the schwannoma and the neurofibroma. The overwhelming benign nature of these lesions necessitates a conservative course of treatment. Histological diagnosis should be followed by a limited tumor excision with emphasis on retaining normal facial nerve function. Malignant lesions require wide excision with facial nerve grafting or facial nerve reanimation.
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ranking = 1
keywords = schwannoma
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6/77. 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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ranking = 1
keywords = schwannoma
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7/77. Bilateral localized orbital neurofibromas.

    BACKGROUND: The authors report on a 30-year-old man who presented with progressive bilateral exophthalmos over a 2-year period. FINDINGS: Computed tomography showed large heterogeneous masses in the superior aspect of both orbits. Excisional biopsy via bilateral lateral orbitotomies showed the tumors to be well-circumscribed, relatively avascular, localized neurofibromas. The patient had several features suggestive of multiple endocrine neoplasia type IIB, including Marfanoid habitus, enlarged corneal nerves, thickened lips, and mucosal neuromas. CONCLUSION: Localized neurofibromas are rare in the orbit and, unlike plexiform neurofibromas, are not typically associated with von Recklinghausen's neurofibromatosis. Bilaterality of such localized neurofibromas has not been previously reported. Recognition of ophthalmic lesions suggestive of multiple endocrine neoplasia IIB should prompt evaluation for systemic manifestations of this disorder.
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ranking = 0.10529608528163
keywords = neuroma
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8/77. Intraneural dendritic cell neurofibroma with pseudorosettes.

    Dendritic cell neurofibroma with pseudorosettes (DCNWPR) is a recently proposed variant of neurofibroma. However, its peripheral nerve sheath origin has subsequently been questioned, and it has been suggested that the neoplasm could represent a hitherto undescribed variant of melanocytic nevus with neural differentiation. Here we report a case of DCNWPR that arose almost exclusively within the confinement of the perineurium in the skin. This observation gives further evidence that this entity is a peripheral nerve sheath tumor and is unrelated to melanocytic neoplasms.
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ranking = 0.15339649819979
keywords = melanocytic
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9/77. Treatment of orbital schwannomas and neurofibromas.

    We present an overview of the treatment and clinical outcome of five orbital peripheral nerve tumours, carried out in our centre from 1999 to 2003. The surgical approach was determined by the location and extension of the lesion. Supraorbital orbitotomy was performed in two superiorly located lesions, a transconjunctival approach in one medial, basal, extraconal lesion. A pterional extradural approach was used in two cases with involvement of the apex, superior orbital fissure and cavernous sinus. Three patients were diagnosed as having schwannoma, one as neurofibroma, and one as cystic mixed neurofibroma and schwannoma. One patient suffered from multiple schwannomas [bilateral acoustic schwannomas, cervical schwannomas (NF2)]. One patient showed bilateral orbital neurofibromas, plexiform cutaneous neurofibroma (NF1) and glaucoma due to a coexisting Marfan's syndrome. Local recurrences were not seen after complete resection in all patients. Surgery is the therapeutic goal.
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ranking = 9
keywords = schwannoma
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10/77. 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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ranking = 6
keywords = schwannoma
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