Cases reported "Neoplasms, Nerve Tissue"

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1/29. Atypical ganglion cell tumor of the sciatic nerve.

    CONTEXT: Although herniation of a lumbosacral intervertebral disk is a major cause of sciatic distribution pain, relentlessly progressive symptoms or signs should alert one to the possibility of a tumor involving the nerve. OBJECTIVE: To describe the clinical, neurophysiological, and histological features of a pathologically unique tumor involving the sciatic nerve. SETTING: Tertiary referral university hospital. PATIENT: A 36-year-old woman was seen with a 6-year history of increasingly severe symptoms in the distribution of the left sciatic nerve. RESULTS: electromyography indicated a sciatic nerve lesion in the region of the greater sciatic notch. magnetic resonance imaging demonstrated a tumor involving the left sciatic nerve in this area. light microscopy, electron microscopy, and immunohistochemistry results confirmed the presence of an atypical ganglion cell tumor of the sciatic nerve that exhibited prognostically conflicting clinical and histological features. CONCLUSIONS: To our knowledge, this is the first report of an atypical ganglion cell tumor affecting the sciatic nerve, and illustrates the value of detailed neurophysiological examination in localizing the site of peripheral nerve injury to facilitate focused neuroimaging when standard investigations are uninformative. Longer follow-up is required to determine the true biologic potential of this lesion.
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2/29. Intraoperative monitoring of an unusual brachial plexus tumor.

    This case illustrates the importance of intraoperative monitoring of neuronal function to help separate tumor tissue from neural tissue in a 54-year-old patient with left shoulder pain resulting from a desmoid tumor. Preoperative nerve conduction and electromyographic studies showed a lesion in the lateral cord of the brachial plexus, which was found to be intimately involved with the tumor mass and was splayed into a very thin effaced sheet of neural tissue. Stimulation of the tumor/nerve tissue mass proximal to the lesion was impossible due to the invasion of the brachial plexus by the tumor. The technique that was adapted for this unusual presentation was to stimulate the tumor/nerve tissue mass itself and record compound muscle action potentials distally. With the technique described, a subtotal resection of an aggressive fibromatosis enmeshed in the proximal brachial plexus was possible, and excellent relief of pain symptoms and retention of functional capabilities of the involved extremity were achieved.
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3/29. Gastrointestinal autonomic nerve tumours: a case report with ultrastructural and immunohistochemical studies.

    A case of gastrointestinal autonomic nerve tumour with light microscopic, immunohistochemical and ultrastructural examination is reported. The tumour was composed of spindle cells or large cells with clear cytoplasm and showed intense staining for vimentin and focal staining for neuron-specific enolase, chromogranin, synaptophysin, gastrin, P substance and S-100 protein. Ultrastructural examination showed long processes with dense core granules and the absence of features characteristic of other gastrointestinal stromal tumours. In addition we noted small traces of basal lamina and the absence of synaptic vesicles. It seems that the biological behaviour of gastrointestinal autonomic nerve tumours is aggressive but there are too few reports on which to conclude anything about their prognosis. Our findings suggest that tumour has a neuroectodermal differentiation.
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4/29. Pancreatic stromal tumor of nerve sheath origin treated by pancreatoduodenectomy.

    A pancreatic sarcoma of nerve sheath origin is reported in a 28-year-old female patient, who presented with melaena. Preoperative imaging showed an 8.5 cm diameter mass in the head of pancreas. There was bleeding from the papilla of Vater at endoscopy and a highly vascular lesion on arteriography. The patient was submitted to proximal pancreatoduodenectomy and remains symptom-free at 1 year follow-up.
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5/29. Formation of nerve twig-like nests and schwannoma in an unusual case of neurofibroma.

    A case of unusual neurofibroma in an 18-year-old Japanese male is reported. The histology of the tumor was characterized by nerve twig-like nests intermingled with fascicular bundles. In the central portion, the tumor also contained a lobular lesion showing features characteristic of schwannoma. Immunohistochemically, the tumor cells in both the nests and the lobular lesion demonstrated a mostly positive reaction for S-100 protein. S-100 protein-positive and -negative cells were observed in equal numbers in the fascicular bundles surrounding the nests. This unusual nerve sheath tumor in which the S-100 protein-positive cells form both nerve twig-like nests and lobular schwannoma has not been reported previously. The origin of the S-100 protein-positive cells in the two lesions is also discussed.
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6/29. Gastrointestinal autonomic nerve tumor.

    The article describes a case of gastrointestinal autonomic nerve tumor, which is histogenetically related to the gastrointestinal autonomic plexus (hence the name plexosarcoma). This rare and only recently recognized tumor of the gastrointestinal tract appears to have significant prognostic implications. This tumor cannot be diagnosed unequivocally by light microscopic and immunocytochemical examinations but shows characteristic electron microscopic features. The present case occurred as a gastric primary tumor and exhibited a light and electron microscopic picture similar to the one described in previous reports: areas of spindle-shaped and epithelioid cells, cytoplasmic processes with dense-core granules, and cytoplasmic intermediate filaments. Ultrastructural characteristics diagnostic of other gastrointestinal tumors, such as those originating from smooth muscle, Schwann cell, or endocrine cell types, were absent. Immunocytochemically, the tumor was diffusely positive for vimentin and neuron-specific enolase and focally positive for neurofilament triplet protein (NFTP) 160. negative staining was observed for NFTP 200, S-100 protein, desmin, somatostatin, chromogranin, keratins (AE1/AE3), and glial fibrillary acidic protein. Although gastrointestinal autonomic nerve tumor has been reported to have a deceptively low-grade malignant appearance by light microscopy, it follows an aggressive clinical course. This tumor showed a much higher mitotic rate (one mitosis per high-power field) than the rates of tumors reported previously. Moreover, it occurred in a much younger patient (20 years of age) compared to previously reported cases (45 to 66 years of age), with the exception of one other case (16 years of age).
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7/29. Malignant peripheral nerve sheath tumor arising in a "de novo" ganglioneuroma. A case report.

    A case of a "de novo" ganglioneuroma showing an internal area of malignant nerve sheath tumor is described. The tumor arose in an 18-year-old girl without a history of von Recklinghausen's disease. Immunohistochemically, the ganglioneuromatous component was positive with anti-synaptophysin, anti-S100 protein and anti-vimentin antisera, whereas the malignant part was immunoreactive only with anti-S100 protein and anti-vimentin antisera. The patient is free of disease 4 years after surgery. The clinicopathologic features of this rare case are discussed.
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8/29. 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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9/29. Painful ophthalmoplegia caused by neurotropic malignant melanoma.

    Progressive cranial nerve palsies and painful ophthalmoplegia developed in a 76-year-old man with lentigo maligna of the forehead 18 months after the excision of a spindle cell tumour of the forehead that proved on immunohistochemical study to be a malignant melanoma. The signs and symptoms were caused by neurotropic spread of the malignant melanoma into the cavernous sinus and subsequent neurosarcomatous transformation of the orbital metastasis.
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10/29. Fine needle aspiration cytology of perineurioma. Report of a case with histologic, immunohistochemical and ultrastructural studies.

    The fine needle aspiration (FNA) findings are presented for a case of perineurioma, a benign soft tissue tumor composed of perineurial cells, which are epithelial-like and ensheath the endoneurial connective tissue space of nerve fibers. A subcutaneous FNA performed on a posterior thigh mass of a 14-month-old boy produced a specimen consisting of numerous spindle-shaped tumor cells with bipolar cytoplasmic extensions and occasional pseudosignet-ring cells in a prominent myxoid background. Cytologically, a diagnosis of benign myxoid tumor was made. Histologic examination of the resected tumor revealed the characteristic features that have been described for perineurioma; the diagnosis was confirmed with immunocytochemical and ultrastructural studies. Perneurioma should be included in the differential diagnosis of benign myxoid neoplasms seen in FNA biopsy specimens.
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