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11/45. Symmetric dumbbell ganglioneuromas of bilateral C2 and C3 roots with intradural extension associated with von Recklinghausen's disease: case report.

    BACKGROUND: Ganglioneuromas are rare benign tumors arising most commonly from the sympathetic nervous system. They occasionally grow in a dumbbell fashion extending into the spinal canal extradurally. However, ganglioneuromas of the cervical spine with intradural extension or multiple locations or in association with von Recklinghausen's disease are rare. CASE DESCRIPTION: A 35-year-old man with von Recklinghausen's disease presented with tetraparesis and respiratory dysfunction. Preoperative neuroimaging revealed an intradural mass extending from the foramen magnum to the C4 vertebral level, as well as bilateral extravertebral extension connecting it with bilateral paraspinal lesions in a dumbbell fashion. Four intradural tumors associated with the bilateral C2 and C3 nerves and located ventrally were removed, leaving the intraforaminal and extradural portion intact. The procedure resulted in postoperative symptomatic improvement. Second, extravertebral tumors of the left neck, which were not related to the cervical sympathetic nerve, were removed. The pathologic diagnosis of the tumors of both the intradural space and cervical neck was ganglioneuroma. CONCLUSION: We present an extremely rare case in an adult with von Recklinghausen's disease who had bilateral, symmetric and multiple dumbbell ganglioneuromas with intradural extension, and also multiple bilateral ganglioneuromas at the neck. The intradural ganglioneuromas were suspected to have originated from the posterior root ganglions of the bilateral C2 and C3 nerves and to have extended ventrally to the spinal cord involving not only sensory but also motor rootlets; the ganglioneuroma of the neck was suspected to have originated from the cervical nerve itself.
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12/45. 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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13/45. Malignant peripheral nerve sheath tumor arising spontaneously from retroperitoneal ganglioneuroma: a case report, review of the literature, and immunohistochemical study.

    We report a case of a malignant peripheral nerve sheath tumor arising from a retroperitoneal ganglioneuroma in an adult male. There was no known previous history of a childhood neuroblastoma or of radiation therapy. In addition, the patient had no stigmata or family history of von Recklinghausen's neurofibromatosis. This case provides evidence that, although rare, spontaneous malignant transformation of ganglioneuroma can occur. Immunohistochemical studies supported that the spindle cell component was of nerve sheath origin.
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14/45. Lobulated schwannoma of the median nerve: pitfalls in diagnostic imaging.

    The authors report an unusual case of volar wrist swelling with the appearance of a simple ganglion on magnetic resonance imaging (MRI); however, operative and histologic examination identified the swelling as an ancient schwannoma. Ancient schwannomas mostly present as a solitary tumor. A lobulated tumor in this location has not been described previously. The pathologic findings of these uncommon tumors and the difficulties encountered in accurate preoperative diagnosis are discussed.
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15/45. November 2004: intradural mass of the cauda equina in a woman in her early 60s.

    November 2004. A 63-year-old woman presented with slowly aggravating lower back pain and recent urinary urge incontinence. MRI revealed a sharply-delineated, partly cystic intradural mass with inhomogenous contrast-enhancement and ectatic vessels at the upper pole. An ependymoma was suspected, and the tumor was resected in toto. Histologically, at first glance, the tumor strongly resembled an ependymoma, showing a monomorphic cellular pattern, perivascular pseudorosettes and ependymal canal-like structures. However, the finding of a delicate collagen capsule, compartmentation of tumor cells into zellballen and the presence of ganglionic cells were untypical. These features were indicative of a paraganglioma with a gangliocytic component. Immunoreactivity of the tumor cells for neuroendocrine antigens, the detection of GFAP-positive sustentacular cells and the ultrastructural confirmation of neurosecretory granules substantiated this diagnosis. The clinical, radiological and morphological similarity between ependymomas, which are far more common in the cauda equina region than paragangliomas, has led to substantial diagnostic confusion in the past.
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16/45. cauda equina tumor with ependymal and paraganglionic differentiation.

    We describe the case of a 31-year-old woman who was first treated for a pigmented choroid plexus papilloma of the fourth ventricle. Ten year later, she developed a new tumor in the region of the cauda equina. This second neoplasm contained areas of papillary ependymoma that displayed phosphotungstic acid hematoxylin-positive glial fibers and immunoreactivity for glial fibrillary acidic and S-100 proteins. Areas of ependymoma merged with others that displayed the appearance of a paraganglioma, including lobules and nests of chief cells immunoreactive for neuron-specific enolase, synaptophysin, chromogranin, and serotonin. Satellite cells, but not chief cells, stained for glial fibrillary acidic and S-100 proteins. Electron microscopy showed features of both ependymal and paraganglionic differentiation, including intercellular lumina with microvilli, junctional complexes, cell processes with closely packed filaments, and dense core granules. Our case represents a rare example of a cauda equina neoplasm with simultaneous ependymal and paraganglionic differentiation. To our knowledge, this is the first described example of a tumor of this region showing features of both ependymoma and paraganglioma.
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17/45. Pigmented ganglioneuroblastoma: relation of melanin and lipofuscin to schwannomas and other tumors of neural crest origin.

    An unusual case of ganglioneuroblastoma containing melanin is presented. Electron microscopy revealed various stages of development of melanosomes in neoplastic cells of Schwann, the first direct demonstration in human material that these cells are malanogenic. The frequent occurrence of neuromelanin in autonomic ganglia and in ganglioneuromas is interpreted as the presence of altered lipofuscin. review of ultrastructural and other observations indicates a relation between various pigmented tumors, the cell of Schwann, and other cells arising from the neural crest.
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18/45. 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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19/45. Hematogenous metastases of carcinoma to dorsal root ganglia.

    Hematogenous metastases of carcinoma to dorsal root ganglia was found in 2 of approximately 500 consecutive autopsies in which a lumbar dorsal root ganglion was routinely examined microscopically. The primary tumors were poorly differentiated colonic adenocarcinoma and oat cell carcinoma of the lung, both with widespread hematogenous metastases which spared the central nervous system. No symptoms were detected clinically. In the same series of patients the sural nerve as well as the lumbar plexus were histologically sampled but no examples of distant endoneurial metastases were found. The vascular endothelium of dorsal root ganglia is fenestrated and, presumably as a consequence, provides no blood-ganglion barrier. This microvascular difference may account for the susceptibility of the ganglia to metastases when compared to nerve trunks which posses unfenestrated endothelium and blood-nerve barrier.
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20/45. 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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