Cases reported "Neurofibroma"

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1/18. Perineal neoplasms.

    Experience with 2 cases of neurally-derived perineal masses is described. A relevant review of the literature spanning 50 years revealed 36 cases of perineal neoplasm of diverse nature, disclosing that such lesions typically present as otherwise asymptomatic enlarging masses and may comprise a broad spectrum of neoplasia with diagnosis and prognosis often relating to the age of the patient. Management naturally relates to tissue type and degree of local involvement.
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2/18. 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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3/18. Nerve sheath tumors involving the sacrum. Case report and classification scheme.

    Nerve sheath tumors that involve the sacrum are rare. Delayed presentation is common because of their slow-growing nature, the permissive surrounding anatomical environment, and nonspecific symptoms. Consequently, these tumors are usually of considerable size at the time of diagnosis. The authors discuss a case of a sacral nerve sheath tumor. They also propose a classification scheme for these tumors based on their location with respect to the sacrum into three types (Types I-III). Type I tumors are confined to the sacrum; Type II originate within the sacrum but then locally metastasize through the anterior and posterior sacral walls into the presacral and subcutaneous spaces, respectively; and Type III are located primarily in the presacral/retroperitoneal area. The overwhelming majority of sacral nerve sheath tumors are schwannomas. Neurofibromas and malignant nerve sheath tumors are exceedingly rare. Regardless of their histological features, the goal of treatment is complete excision. Adjuvant radiotherapy may be used in patients in whom resection was subtotal. Approaches to the sacrum can generally be classified as anterior or posterior. Type I tumors may be resected via a posterior approach alone, Type III may require an anterior approach, and Type II tumors usually require combined anterior-posterior surgery.
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4/18. A case of bathing trunk nevus studded with neurofibroma-like papules.

    There is a significant association between bathing trunk nevus and neurofibromatosis. However, not all neurofibroma-like papules detected clinically in cases with bathing trunk nevus may be a neurofibromas and histopathological confirmation is essential for definition of these lesions. We report a 21 year old white male patient with a bathing trunk nevus studded with neurofibroma-like papules of melanocytic nature. Histopathological examination of both papular and flat lesions showed diffuse melanocytes with plentiful eosinophilic cytoplasm and round nuclei in the dermis. immunohistochemistry showed strong positivity of dermal melanocytes for S-100 protein. The clinical and histopathological findings and positivity of dermal melanocytes for S-100 protein were consistent with bathing trunk melanocytic nevus.
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5/18. Nerve tumors of the hand and forearm.

    Peripheral nerve tumors comprise less than 5% of all tumors of the hand. The most common solitary nerve tumor is the neurilemmoma, which arises from the neural sheath, is well encapsulated, minimally symptomatic, and may be surgically enucleated without producing a neurological deficit. Neurofibromas may be solitary, multiple, or associated with von Recklinghausen's disease. They are usually centrally placed with nerve fibers traversing the tumor mass making it more difficult to remove the tumor without producing permanent neurological damage. Malignant tumors include neurofibrosarcomas which often are very aggressive, requiring wide excision or amputation, and the rare neuroepitheliomas. Reported nerve tumors, intraneural in location but nonneural in origin, include fibrofatty infiltration of the median and digital nerves, intraneural lipoma, hemangioma, and ganglion cysts. These lesions may be treated by decompression or excision, depending on the nature of the tumor. Four unusual cases are described.
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6/18. Neurogenic sarcoma of the sinonasal tract.

    Five cases of a rare malignant sinonasal tumour of neurofibromatous nature are described. The term neurogenic sarcoma (or malignant peripheral nerve sheath tumour) is advocated instead of malignant schwannoma. The clinicopathological features of this tumour are described, and the different diagnosis are discussed. The necessity of using immunohistochemistry in the diagnosis of sinonasal tumours of fibromatous nature is emphasized. The clinical management consists of radical surgery and adjuvant radiotherapy. Clinically there seems to be a high-grade and a low-grade type of sinonasal neurogenic sarcoma, however, this could not be distinguished histologically in the present series.
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7/18. The occurrence of different types of spinal tumours in one patient. A case report and review of the literature.

    Three histologically different types of spinal tumours were found in a 16-year old girl. Each tumour was detected independently at a distant region. They were two intramedullary ependymomas at C3 and T2, two schwannomas at the C6 and C7 root, and one intradural meningioma at T7. All tumours were successfully removed by a two-stage operation without adding neurological deficits. In this paper the findings of metrizamide myelography, metrizamide CT, intravenous enhanced CT and MRI are presented. The application of these various neuroradiological methods made it possible to confirm the precise location and the nature of the tumour in order to operate safely.
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8/18. Multiple cytogenetic aberrations in neurofibrosarcomas complicating neurofibromatosis.

    Four neurofibrosarcomas, from four unrelated patients with Von Recklinghausen's neurofibromatosis, demonstrated multiple cytogenetic aberrations characterized by loss of chromosomes and/or polysomy, as well as multiple structural abnormalities of diverse types. These observations are noteworthy for two reasons. First, the widespread and varied nature of the chromosomal changes indicate that, in at least these instances, the transformation of a benign neurofibroma to a neurofibrosarcoma may well involve a substance having an affect at multiple chromosomal sites. Second, efforts to investigate neurofibrosarcoma pathogenesis using direct analysis of dna structure and gene activity in neurofibrosarcomas must first take into account the chromosomal distortions characterizing each tumor.
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9/18. Craniocervical manifestations of neurofibromatosis: MR versus CT studies.

    Craniocervical manifestations of neurofibromatosis on magnetic resonance (MR) imaging are described in three patients and compared with those on CT. Using MR, intracranial gliomas, schwannomas, and neurofibromas were detected as well as with CT. In addition, a brain stem lesion that was not visualized on CT and a cervical cord lesion that was not suspected clinically were diagnosed with MR. Taking into account the noninvasive nature of MR, we believe that this technique should be the primary imaging modality for screening and follow-up studies in patients with known or suspected neurofibromatosis.
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10/18. Giant appendicular neurofibroma. A light and immunohistochemical study.

    A case of solitary appendicular neurofibromatosis is presented. The neurogenous nature of the lesion was emphasized by the positive reaction for S-100 protein. attention is directed towards the morphological differences between neurogenous hyperplasia, which is a reactive common place feature in the elderly, and the present example of neoplastic nerve growth, which produced a gigantic appendix. A review of the literature failed to disclose any previous case unassociated with von Recklinghausen's disease.
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