Cases reported "Head and Neck Neoplasms"

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1/51. E.N.T. manifestations of Von Recklinghausen's disease.

    Von Recklinghausen's disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.
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keywords = neurofibroma
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2/51. Malignant peripheral nerve sheath tumor arising in benign ancient schwannoma: a case report with an immunohistochemical study.

    A rare example of malignant transformation in an ancient schwannoma arising in the right side of the neck of a 51-year-old man without any clinical manifestations suggesting neurofibromatosis is described. The tumor, approximately 4 cm at its largest dimension, was well circumscribed and had a direct connection with the sympathetic nerve. Microscopically, the central portion of the tumor showed features of ancient schwannoma characterized by extensive hyalinization with cystic degeneration, scattered spindle cells with hyperchromatic and tapered nuclei, and some symplastic changes. However, predominantly in the outer portion, a proliferation of spindle-shaped cells with enlarged nuclei was present. The nuclei of these cells showed irregular contours, coarse granular chromatin texture, and conspicuous nucleoli. Mitotic figures and small necrotic foci with scattered apoptotic bodies were also seen. Immunohistochemically, S-100 protein was almost negative in areas consisting of overtly atypical cells where the mitotic index evaluated with MIB-1 antibody was 30.5%. In contrast, S-100-positive bland spindle cells were scattered in an extensively hyalinized area with a labeling index less than 3%. P53 protein was strongly positive in atypical spindle cells. Although it is a very uncommon event, definite nuclear atypia, frequent mitotic figures, and the existence of small necrotic foci should be recognized as indicating a diagnosis of malignant degeneration of benign schwannoma. immunohistochemistry would be useful as an ancillary technique in such a setting.
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ranking = 0.33333333333333
keywords = neurofibroma
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3/51. Glandular malignant peripheral nerve sheath tumor: an unusual case showing histologically malignant glands.

    In this report, we describe a highly unusual case of glandular malignant peripheral nerve sheath tumor presenting as a neck mass in a previously healthy 29-year-old man. Grossly, the tumor was found to arise from a swollen peripheral nerve trunk. The tumor was largely composed of spindle cells that demonstrated marked nuclear pleomorphism and numerous abnormal mitotic figures. In addition, histologically malignant glandular structures lined by simple nonciliated columnar cells with goblet cells were found clustered in the center of the tumor. Examination of the swollen peripheral nerve trunk revealed the presence of a plexiform neurofibroma. The spindle cells were positive for S100. The glands were negative for S100 but positive for keratin, epithelial membrane antigen, and neuroendocrine markers (somatostatin, chromogranin, Leu-7, and calcitonin). This patient was subsequently diagnosed as having von Recklinghausen disease and died of tumor metastasis to the lungs 34 months after the presentation. To our knowledge, only 3 similar cases have been previously described in the literature.
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ranking = 0.33333333333333
keywords = neurofibroma
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4/51. Bilateral posterolateral approach to mirror-image C-2 neurofibromas. Report of four cases.

    Multiple nerve root tumors are usually present in patients afflicted with neurofibromatosis Type 1. Although rare, upper cervical mirror-image neurofibromas have been reported in the medical literature, and their surgical management has been addressed in several reports; however, little has been mentioned or is known regarding upper cervical or craniocervical stability following resection of these tumors. In this report the authors describe four cases of large mirror-image C-2 neurofibromas resected in two stages via the posterolateral approach. One patient presented with acute neurological deterioration after a biopsy sample had been obtained, whereas the other three presented with gradual onset of lower-extremity weakness over several months. The time interval between the first and second decompressive surgery ranged from 10 days to 12 weeks. There were no surgery-related complications, and all patients recovered motor function in their extremities. During a follow-up period of 16 to 36 months, there was no clinical or radiological evidence of upper cervical spine instability. Although the series is too small to draw any definitive conclusions, in the authors' experience the posterolateral approach provides a direct route for the successful surgical treatment of bilateral craniocervical nerve root tumors without destabilizing the upper cervical segments.
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ranking = 2.3333333333333
keywords = neurofibroma
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5/51. Neurofibromatosis type 1 of the head and neck: dilemmas in management.

    Neurofibromatosis type 1 (NF 1) or Von Recklinghausen's disease is an autosomal dominant condition characterized by multiple skin cafe-au-lait lesions and multiple neurofibromas. In the head and neck neurofibromas have a predilection to arise in th deep planes of the neck. Surgical management is nearly always required for functional or cosmetic reasons or to exclude the possibility of malignant transformation. We present four cases of neurofibroma of the head and neck and illustrate the difficulties inherent in managing this condition, particularly the age range in which it may occur, the risk of malignant transformation, the possibility of abnormal bleeding and the morbidity that may be associated with the surgical resection. The role of magnetic resonance (MR) scan in the assessment and monitoring of these lesions is highlighted. We also report a unique association of a pharyngeal pouch with NF 1 in a young (25-year-old) patient.
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keywords = neurofibroma
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6/51. 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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ranking = 2.3333333333333
keywords = neurofibroma
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7/51. Superficial malignant schwannoma of the scalp.

    BACKGROUND: Malignant schwannoma is a rare sarcoma that is located mostly on trunk and extremities; the head and neck are unusual sites for its development. Almost half of these tumors arise from neurofibromas with or without von Recklinghousen's disease, and most of the remainder develop de novo from peripheral nerve trunks. Development in the superficial soft tissues is extremely rare. OBJECTIVE: To present a case of superficial malignant schwannoma of the scalp, which is an uncommon presentation. methods: An 80-year-old male patient presented with a painless ulceration on his scalp. The clinical and radiologic properties of the tumor were consistent with a skin tumor. RESULTS: The lesion was excised, and reconstruction was achieved by transposition scalp flap. Malignant schwannoma was given as diagnosis by histopathologic examination with immunohistochemistry. CONCLUSION: Malignant schwannomas may also occur in the skin. Clinically, these superficial forms closely resemble skin tumors, especially malignant melanoma. The treatment of choice in these tumors is wide resection combined with radiotherapy.
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ranking = 0.33333333333333
keywords = neurofibroma
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8/51. Massive plexiform neurofibroma with associated meningo-encephalocoele and occipital bone defect presenting as a cervical mass.

    The commonest skull manifestations in neurofibromatosis involve the orbit, with very few reports about occipital defects. We report a rare case of a 54-year-old lady with a massive plexiform neurofibroma extending from the auricular region down her left neck and into her shoulder, with an associated large left occipital and left petrous bone defect and extensive cerebellar meningo-encephalocoele, which presented with a relatively asymptomatic cervical mass and was treated with resection of the neurofibroma and advancement and rotational skin flaps.
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ranking = 2.3333333333333
keywords = neurofibroma
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9/51. 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 = 0.33333333333333
keywords = neurofibroma
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10/51. Perineurioma: an uncommon soft tissue neoplasm distinct from localized hypertrophic neuropathy and neurofibroma.

    Perineurial cells, which normally surround the nerve fascicles within a nerve, can be distinguished from schwann cells by their immunoreactivity for epithelial membrane antigen (EMA) and lack of reactivity for S-100 protein. We report two cases of perineurioma, a tumor composed exclusively of perineurial cells and distinct from other nerve sheath tumors. The first case involved a deep, soft-tissue mass of the neck, and the second involved a tumor located in the infraclavicular subcutaneous tissue. Both tumors were well circumscribed. Histologically, they were hypocellular and composed of spindle cells possessing elongated nuclei and bipolar, wavy, slender, strikingly elongated cytoplasmic processes, disposed in a background of collagen in the form of short bundles and whorls. In the first case, there were frequent calcospherites and remnants of a small nerve at the periphery. The spindle cells stained for EMA but not S-100 protein, chromogranin, neuron-specific enolase or Leu-7. Ultrastructurally, they possessed long cytoplasmic processes with incomplete basal lamina and occasional pinocytotic vesicles. axons were not identified. review of the literature shows that genuine perineuriomas are rare, and most cases reported as such are merely examples of localized hypertrophic neuropathy, a mononeuropathy characterized by fusiform swelling of a nerve, usually in the extremities. The involved segment in localized hypertrophic neuropathy contains distended fascicles composed of whorls of perineurial cells and fibrous tissue entrapping residual axons, probably representing a hyperplastic reaction to nerve damage. The term perineurioma should be reserved for the neoplasm composed only of perineurial cells and presenting as a soft tissue tumor.(ABSTRACT TRUNCATED AT 250 WORDS)
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ranking = 1.3333333333333
keywords = neurofibroma
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