Cases reported "Neurofibroma"

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1/55. neurofibroma in the median nerve treated with resection and free nerve transplantation. case reports.

    The commonest tumours of the peripheral nerves are neurolemomas and neurofibromas, both arising from the nerve sheath. Both tumour types are described. It is established that the neurofibroma becomes malignant in about 10% of cases and that removal involves resection of the nerve. Radical resection is nevertheless recommended and the defect in the nerve may be overcome by free nerve transplantation. Two cases are reported.
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ranking = 1
keywords = peripheral nerve, peripheral
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2/55. A corneal diffuse neurofibroma as a manifestation of von recklinghausen disease.

    PURPOSE: To report a case of a primary corneal diffuse neurofibroma in a patient with von Recklinghausen disease (NF-1). methods: Case report. A physical examination and histopathology were performed. The immunohistochemical studies were performed using an avidin-biotin-peroxidase complex technique on formalin-fixed and paraffin-embedded tissue. Histologic sections from corneal tissue were incubated with primary antibodies against vimentin and S-100 protein. A complementary ultrastructural study of the same formalin-fixed and paraffin-embedded tissue was made. RESULTS: The ophthalmologic examination revealed a yellowish-white elevated mass that involved the supratemporal cornea but not the limbus. Histologic study showed a tumor of the peripheral nerve sheath, a diffuse neurofibroma in the corneal stroma, and proliferation of spindle cells with markedly elongated nuclei. Cells comprising the tumor reacted with vimentin and S-100 protein, and the ultrastructural studies revealed myelinated nerve fibers confirming the diagnosis. CONCLUSION: The development of a primary diffuse neurofibroma in the cornea of patients with von Recklinghausen disease is possible. The present case supports the statement that neurofibromas arising from the peripheral nerve sheath may involve any part of the body.
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ranking = 2
keywords = peripheral nerve, peripheral
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3/55. 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 = 2
keywords = peripheral nerve, peripheral
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4/55. 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 = 1
keywords = peripheral nerve, peripheral
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5/55. Case studies for illustration and discussion: peripheral nerve tumors.

    This article gives a presentation of a variety of surgical cases of peripheral nerve tumors for illustration and discussion. The first three cases include a schwannoma, a neurofibroma, and a desmoid tumor of the brachial plexus region. Case 4 is that of a patient with a common peroneal ganglion cyst, and case 5 is that of a patient with an angiolipoma of the forearm. Cases 6 through 8 illustrate a plexiform neurofibroma, a malignant peripheral nerve sheath tumor, and a metastatic carcinoma to the brachial plexus region. In case 9, a patient with schwannomatosis was treated for multiple spinal schwannomas.
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ranking = 6
keywords = peripheral nerve, peripheral
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6/55. Severe haemorrhage in neurofibromatoma: a lesson.

    Neurofibromatosis is a relatively common inherited disease of the nervous system, with a frequency of almost 1 in 3000. It is associated with a wide range of vascular abnormalities. A 62-year-old man with neurofibromatosis presented to us with a sacral haematoma. This was due to spontaneous rupture of a pre-existing neurofibromata. Upon admission the patient was in hypovolaemic shock and required aggressive resuscitation prior to surgery. Haemorrhage following trauma or spontaneous rupture is an uncommon complication of neurofibromatosis. The management is discussed with emphasis on the cause and control of bleeding from these lesions.
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ranking = 0.00039117112709085
keywords = nervous system
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7/55. Supraclavicular approach without thoracotomy for posterior mediastinal tumor.

    Using a right-sided supraclavicular approach, we operated on a 15-year-old girl with a posterior mediastinal neurogenic tumor and von Recklinghausen's disease. The encapsulated 95 x 65 x 40 mm neurofibroma was removed safely and completely without thoracotomy. No peripheral neurologic defect such as Horner's syndrome, paralysis of accessory nerve, or palsy of the right upper extremity was observed after operation. The postoperative course was uneventful and the patient was discharged on the third postoperative day with no symptoms. Today, various adaptations of video-assisted thoracic surgery are frequently utilized for many lesions. However, more direct, safer, and less invasive approaches should be selected based on local anatomy, such as the supraclavicular approach without thoracotomy that we utilized.
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ranking = 0.075854968714042
keywords = peripheral
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8/55. Comparative ultrastructural and immunohistochemical study of perineurioma and neurofibroma of the oral mucosa.

    In the course of assessing the cellular composition of intraoral neurofibroma (NF), we encountered a unique gingival tumor of putative perineurial (PN) origin. The lesion showed the ordinary light microscopic NF pattern, but the ultrastructural features of well-differentiated PN cells as well as an epithelial membrane antigen (EMA)-positive, S-100 protein-negative immunoprofile confirmed the diagnosis of soft tissue perineurioma (STP). In our small series of NF, there were three ultrastructural subtypes: Type I (common Schwann cell type), Type II (NF with a high content of PN cells) and Type III (predominantly fibroblastic NF), although inhomogeneous and overlapping assembly of cellular elements. A significant number of tumor cells in Type II showed the substantial reactivity for EMA, whereas many CD34-positive cells were noted in Type III. The present results confirm previous findings that PN lineage is an important constituent in the formation of NF and reinforce the value of electron microscopy in the diagnosis of peripheral nerve sheath tumors.
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ranking = 1
keywords = peripheral nerve, peripheral
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9/55. Hybrid peripheral nerve sheath tumors: Schwannoma-perineurioma and neurofibroma-perineurioma. A report of three cases in extradigital locations.

    We present three cases of subcutaneous tumors with hybrid features of schwannoma-perineurioma (one case) and neurofibroma-perineurioma (two cases), which occurred in two women aged 50 and 52 years and one man aged 52. Locations included the scapular area, skin overlying breast and knee area. The tumors were 1.5, 4 and 5 cm in largest diameter. None of the patients had signs of neurofibromatosis. All tumors were surgically removed, and patients remained disease-free for 1 to 4 years. The classification of the lesion into schwannoma-perineurioma and neurofibroma-perineurioma rested on histopathological and immunohistochemical findings. An ultrastructural study was performed in one case of neurofibroma-perineurioma. All cases were studied for mutation of the NF2 gene, and in one case (neurofibroma-perineurioma) a point mutation was detected in exon 15 of the gene.
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ranking = 4
keywords = peripheral nerve, peripheral
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10/55. Biallelic somatic inactivation of the NF1 gene through chromosomal translocations in a sporadic neurofibroma.

    neurofibroma is a benign tumor originating from schwann cells in peripheral nerve sheaths and may occur as a sporadic tumor or as part of the dominantly inherited tumor syndrome NF1. NF1 is caused by constitutional mutations in the NF1 gene, located in chromosome band 17q11. Whereas the involvement of the NF1 gene in neurofibroma development in NF1 patients has been fairly well characterized, the significance of inactivation of this gene in sporadic neurofibromas remains less well investigated. Inactivation of both copies of NF1 has been described in a few neurofibromas from NF1 patients, and LOH at the same locus has been reported in additional cases. In the present study, we report the cytogenetic and molecular cytogenetic findings in a sporadic neurofibroma that at G-banding analysis showed a translocation between one chromosome 2 and the long arms of both copies of chromosome 17. FISH analysis using a set of 3 BAC clones covering the entire coding region of NF1 revealed the complete loss of one allele and the deletion of the 5' portion of the second allele as a result of 2 translocation events. To the best of our knowledge, this represents the first demonstration of a somatic biallelic inactivation of the NF1 gene in neurofibroma, providing further evidence for the importance of NF1 inactivation also in sporadic neurofibromas.
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ranking = 1
keywords = peripheral nerve, peripheral
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