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1/58. Primary non-Hodgkin's lymphoma of brachial plexus.

    We report the case of a 65-year-old man with non-Hodgkin's lymphoma (NHL) not only in the brachial plexus but also in the central nervous system and parotid gland. He was referred to our hospital for evaluation of a right parotid mass. He also presented with bilateral facial palsy and paralysis of the left superior limb. Computed tomography scan and magnetic resonance imaging revealed mass lesions in the right parapharyngeal space, the deep lobe of the right parotid gland. and the left brachial plexus. A gallium-67 citrate scan demonstrated abnormal uptake in the left brachial plexus. These symptoms and lesions improved during steroid therapy. However, the symptoms worsened again after steroid therapy was discontinued. We performed a right parotidectomy to confirm the diagnosis. Histopathological study revealed NHL. He was treated with combination chemotherapy, and most of the lesions and symptoms, except bilateral facial palsy, improved. Despite follow-up treatment, a brain metastasis occured, and he died 16 months after the onset of symptoms.
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keywords = nervous system
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2/58. Solitary sciatic nerve lymphoma as an initial manifestation of diffuse neurolymphomatosis. Case report and review of the literature.

    Solitary peripheral nerve lymphomas are exceedingly rare primary manifestations of diffuse peripheral nervous system or central nervous system (CNS) lymphomatosis. A 52-year-old man presented with progressive weakness in gastrocnemius and anterior tibial muscle function, which was associated with radiating pain in the right leg. magnetic resonance imaging studies revealed a solitary fusiform tumor, extending from the sciatic nerve, at the level of the lesser trochanter of the femur, into the posterior tibial nerve below the popliteal fossa. Intraoperative gross examination found that the tumor diffusely expanded the nerve, but did not extend from or into surrounding muscle or tendons. The final histological diagnosis was a solitary extranodal lymphoma (Burkittlike high-grade B-cell lymphoma). Postoperative staging did not reveal evidence of lymphomatous involvement of other organs, but additional chemo- and radiotherapies were administered. Four months after the surgical biopsy, the patient presented with a right facial nerve palsy. The results of cytological examination of cerebrospinal fluid were positive for the presence of atypical lymphocytes, which was consistent with apparently progressive neurolymphomatosis; however, the results of radiological studies were negative for systemic progression. The patient underwent intrathecal chemotherapy followed by systemic myelosuppressive chemotherapy with bone marrow rescue, but died of respiratory failure while still receiving treatment. Postmortem examination revealed extensive lymphomatosis in the peripheral nerves and spinal nerve roots without evidence of cranial nerve, CNS, or other organ system involvement. The aggressive biological characteristics of these tumors, their management, and pertinent literature are reviewed.
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keywords = nervous system
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3/58. paraganglioma of the cauda equina. Case report with 33-month recurrence free follow-up and review of the literature.

    paraganglioma of the cauda equina is an unusual tumor and do not have the secretory properties of the same tumors arising outside the nervous system. In none of the few cases reported in literature a preoperative diagnosis was possible, and the surgical findings raised questions in the differential diagnosis with ependymomas. A rare case of paraganglioma of the cauda equina studied both pre- and postoperatively by MRI, and treated with subtotal excision combined with radiotherapy is described. Results and recurrence rates of the cases reported in literature are reviewed. Though MRI imaging has proven to be more sensitive than other radiological procedures, we stress the difficulties of preoperative diagnosis of paragangliomas in this site. The correct diagnosis of the paraganglioma of the cauda equina still relies on immunochemistry and electron microscopy. Total excision is often very difficult owing the tendency of these neoplasms to infiltrate cauda's roots. A 33-month recurrence free follow-up of our patient confirms that successful treatment is achieved by subtotal resection combined with radiotherapy.
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keywords = nervous system
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4/58. Primary lymphoma of peripheral nerve: report of four cases.

    lymphoma presenting as a solitary tumor of peripheral nerve is exceedingly rare, with only six previously reported cases. The authors describe an additional four cases of primary lymphoma of peripheral nerve involving the sciatic nerve (two cases), the radial nerve, and the sympathetic chain and spinal nerve. The patients were two men and two women with an average age of 55.5 years. All tumors were high-grade B-cell lymphomas. Two patients experienced relapse of disease with involvement of other nervous system sites and died of lymphoma. One patient is alive with stable local disease at 57 months. The fourth patient is alive with no evidence of disease at 54 months. Expression of neural cell adhesion molecule (CD56) has been reported to correlate with an increased incidence of central nervous system involvement in peripheral T-cell lymphoma; all their cases were CD56 negative. Recent reports indicate a high proportion of primary brain lymphomas show loss of CDKN2A/p16 gene expression. Therefore, CDKN2A/p16 was evaluated in their patients both by polymerase chain reaction and by immunohistochemistry for the p16 protein. The authors found homozygous deletion of the CDKN2A/p16 gene in one of three patients studied, confirmed immunohistochemically by absent staining for p16. The fourth patient showed absent staining for p16, suggesting inactivation of the gene in this case as well. The two patients with p16 loss both died of lymphoma, whereas the two patients with normal p16 expression are alive. Primary lymphoma of peripheral nerve is a rare neoplasm, usually of large B-cell type, has a variable prognosis, and appears to have less consistent loss of p16 expression than primary central nervous system lymphoma.
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keywords = nervous system
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5/58. Primary primitive neuroectodermal tumor of the cauda equina.

    Primitive neuroectodermal tumors (PNETs) are aggressive neoplasms composed predominantly of undifferentiated cells that show evidence of neural differentiation. Although their classification has been controversial, PNETs are well recognized primary tumors of both central and peripheral nervous systems. PNETs must be distinguished from other round-cell tumors, including Ewing's sarcoma, lymphoma, rhabdomyosarcoma, and small cell carcinoma. Intraspinal PNETs are rare neoplasms that are usually metastatic in origin. We describe the eighth reported primary PNET of the cauda equina that developed in a 52-year-old man with no significant medical history. The tumor was characterized by Homer-Wright rosettes and immunoreactivity for CD99, glial fibrillary acidic protein, neuron-specific enolase S100, and synaptophysin. The anatomic location of primary intrathecal PNETs is important as those arising in the spinal cord develop in the central nervous system, whereas those arising in the cauda equina develop in the peripheral nervous system. The histogenesis of intrathecal PNETs may be multifactorial.
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ranking = 3
keywords = nervous system
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6/58. Haemangioblastoma of a cervical sensory nerve root in Von Hippel-Lindau syndrome.

    Spinal haemangioblastomas are rare, accounting for only about 7% of all central nervous system cases. The case of a 40-year-old woman with a haemangioblastoma arising solely from a cervical sensory nerve root is presented. At operation via a cervical laminectomy, it was possible to resect the tumour en masse with the sensory ramus, by extending the laminectomy through the exit foramen for C6. Haemangioblastomas are commonly intramedullary, and have only been reported in this location on one previous occasion. The patient has Von Hippel-Lindau syndrome and a history of multiple solid tumours. The possible role of the Von Hippel-Lindau tumour suppressor gene in the pathogenesis of these neoplasms is discussed.
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keywords = nervous system
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7/58. Intraneural nerve metastasis with multiple mononeuropathies.

    Although cancer is a frequent condition, neoplastic involvement of the peripheral nervous system is rare. The mechanisms are heterogeneous and include lesions within the cerebrospinal fluid (CSF) space, local invasion (e.g. brachial plexus), compression, rarely direct infiltration, perineurial spread and even rarer intranerval metastasis. A 47-year-old woman had been treated for a carcinoid 10 years earlier and had received axillar irradiation. At presentation she suffered from weakness of the biceps brachii and was experiencing pain radiating from the axilla into the forearm and thumb. MR scans of the brachial plexus were negative and her symptoms were primarily considered to stem from a postradiation brachial plexopathy, Because of increasing pain, the brachial plexus was explored and a metastasis in the left musculocutaneous nerve was resected. Several months later, numbness and pain appeared in the ulnar nerve and another intrafascicular metastasis in the ulnar nerve was discovered. Resection with preservation of remaining fascicles was performed. This rare case report demonstrates that multiple mononeuropathies, resembling multiplex neuropathy, may be caused by intranerval metastasis.
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8/58. Primary intrathoracic meningioma: histopathological, immunohistochemical and ultrastructural study of two cases.

    Meningiomas are common, usually benign slow-growing neoplasms of the central nervous system thought to arise from meningocytes capping arachnoid villi. Primary ectopic meningiomas are exceedingly rare extracranial and extraspinal tumors of controversial origin; they are usually limited to the head and neck region or to the paravertebral soft tissues. Only one mediastinal ectopic meningioma and few pulmonary ectopic meningiomas have been described in the literature until now. Because of their rarity and their intriguing pathogenesis, we report here a second case of primary mediastinal meningioma and an additional case of primary pulmonary meningioma. Their possible origin and differential diagnosis are discussed.
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keywords = nervous system
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9/58. Negative-antibody paraneoplastic syndrome complicating small cell carcinoma.

    The neurological paraneoplastic syndromes represent nonmetastatic complications of cancer and may affect several levels of the nervous system. They are thought to be immunologically-mediated. The syndrome predates the diagnosis of cancer by months to years in two thirds of cases. We report the case of a female patient presenting with a cerebellar syndrome and a sensory neuronopathy on a background of severe weight loss. We searched for occult malignancy and later diagnosed her to be suffering from a paraneoplastic syndrome secondary to small cell carcinoma of the lung. Paraneoplastic antibodies were negative. She was subsequently treated with chemotherapy.
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keywords = nervous system
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10/58. Pilocytic astrocytoma of a spinal nerve root. Case report.

    A case of pilocytic astrocytoma involving a spinal nerve root is reported. A 39-year-old woman presented with a 1-year history of progressive pain and numbness, predominantly in the S-1 dermatome. Magnetic resonance (MR) imaging revealed an intradural lesion at the tip of the conus medullaris. The intradural tumor was excised as was the sacrificed nerve root. Histological examination showed a pilocytic astrocytoma in which there were unusual features of calcification and ossification. At 3-year follow-up review MR imaging demonstrated no residual tumor. To the best of the authors' knowledge, this is the first case of a primary pilocytic astrocytoma, a tumor typically of central nervous system origin, arising from a spinal nerve root.
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