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1/152. Gigantic benign schwannoma in the lateral peroneal nerve.

    While schwannomas of the lateral peroneal nerve at the neck of the fibula are rare, this entity should be considered in the differential diagnosis of popliteal cysts and in all cases of pain or paresthesia of the leg and foot. magnetic resonance imaging is the diagnostic tool of choice for diagnosis of schwannoma.
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2/152. Solitary schwannoma of the cervical vagus nerve.

    A case of benign, solitary schwannoma of the cervical vagus nerve with ipsilateral vocal cord paralysis is presented. The differential diagnostic aspects are discussed, with special reference to neurologic deficit in association with this lesion. The possible occurrence of concomitant unrelated malignancy is emphasised.
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3/152. Axillary schwannoma masquerading as cervical radiculopathy.

    A 58-year-old woman, suffering from radicular-like pain in the left arm for 3 years, presented an entirely negative cervical imaging. Careful clinical examination disclosed Tinel's sign in the axilla. This clinical finding led to further investigation of this region. Computed tomography and magnetic resonance imaging disclosed a small tumour highly suggestive of a schwannoma. Surgical exploration and microscopic examination confirmed a diagnosis of schwannoma located on the radial trunk of the left brachial plexus. After tumour excision, the patient had immediate relief of pain without sensitive or motor sequelae. No recurrence has been observed after 3 years.
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4/152. Molecular analysis of malignant triton tumors.

    Triton tumors are rare variants of malignant peripheral nerve sheath tumor (MPNST) with muscle differentiation, often seen in patients with neurofibromatosis 1 (NF1). Individuals affected with NF1 harbor mutations in the NF1 tumor suppressor gene and develop neurofibromas and MPNSTs. The NF1 gene is expressed in schwann cells and its expression is lost in schwannian neoplasms, suggesting a role in malignant development. Separately, there is evidence that p53 suppressor gene mutations are involved in MPNSTs. To determine the role of the NF1 and p53 genes in the development of the malignant Triton tumor we examined 2 such tumors, 1 from a 3-year-old boy without clinical manifestations of NF1 and another from a 24-year-old man with NF1. Histological analysis of these tumors showed both neural and muscle differentiation with S-100 and desmin immunoreactivity, respectively. Reverse transcribed rna polymerase chain reaction (RT-PCR) of NF1 mRNA showed NF1 expression in the sporadic tumor. Strong nuclear immunoreactivity for p53 was observed throughout the malignant population in both tumors. This was confirmed by loss of heterozygosity for p53 in the non-NF1 patient, suggesting that p53 is involved in both hereditary and sporadic Triton tumors. The finding of preserved NF1 gene expression in the non-NF1-related Triton tumor suggests that different genetic events predispose to the development of this rare neoplasm in sporadic cases.
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5/152. Macrocystic schwannoma in the seventh cervical nerve.

    We have recently treated a case of asymptomatic macrocystic schwannoma discovered between the left neck and the supraclavicular fossa. The tumor, originating from the left seventh cervical nerve, was extirpated. Nearly 70 percent of the tumor area showed macrocyst with transparent fluid collection. The tumor was diagnosed as macrocystic schwannoma histopathologically. Schwannoma is often associated with small cysts but rarely with macrocysts. Because schwannoma is a benign tumor, partial resection against an attempt at total removal resulting in progressive neurologic deficits is recommended. In general, schwannoma does not present any apparent symptoms at an early stage, because it occurs and progresses very slowly. Schwannoma originating at the spinal nerve root has been called a dumbbell-shaped tumor because of its characteristic shape on MRI and cystogram images. We recently have treated a case of dumbbell-shaped tumor that was an asymptomatic macrocystic schwannoma in the seventh cervical nerve.
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6/152. Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases.

    AIMS: Angiosarcomatous differentiation represents the least common form of heterologous differentiation in malignant peripheral nerve sheath tumours (MPNST), and is seen most frequently in patients with neurofibromatosis type 1. More rarely, it has been reported in patients without stigmata of neurofibromatosis, or in benign nerve sheath tumours and peripheral nerves. This study was undertaken to confirm this rare association. methods AND RESULTS: Four cases of angiosarcoma arising in a peripheral nerve, in a long-standing schwannoma and in two MPNST are described. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. An intraneural high-grade epithelioid angiosarcoma arose in the left posterior tibial nerve of a 78-year-old man, a well to moderately differentiated angiosarcoma was seen in an ancient schwannoma of the lateral neck in a 73-year-old women, and an angiosarcoma of varying grades of differentiation developed in a recurrent MPNST in the thigh of 38-year-old man. In addition a high-grade MPNST in the axillary region of a 30-year-old man showed foci of heterologous high-grade angiosarcomatous differentiation. The neural and endothelial lines of differentiation were confirmed in each case by positive immunohistochemical staining for neural and endothelial markers, respectively. In all cases tested, the neural differentiated cells stained immunohistochemically positive for antibodies against vascular endothelial growth factor. CONCLUSIONS: This study confirms the rare association of angiosarcoma arising in peripheral nerves, as well as in benign and malignant peripheral nerve sheath tumours.
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7/152. Schwannoma of the cervical sympathetic chain: it's not a carotid body tumor.

    Schwannoma of the cervical sympathetic chain is a rare nerve tumor with fewer than 40 confirmed cases in the English literature. These lesions typically present as an asymptomatic neck mass and are easily mistaken for a carotid body tumor during the initial workup. We report a case of schwannoma of the cervical sympathetic chain in a 35-year-old man followed by a review of the current literature.
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8/152. Giant cauda equina schwannoma. A case report.

    STUDY DESIGN: Case report. OBJECTIVES: To present a rare case of a giant schwannoma of the cauda equina. SUMMARY OF BACKGROUND DATA: Giant spinal schwannoma of the cauda equina, which involves many nerve roots, is rare and there is usually no ossification in the schwannoma. It is unknown whether or not complete excision is preferable if the tumor is located in the lumbar lesion. methods: A 57-year-old woman had a 10-year history of low back pain. Scalloping of the posterior surface of the vertebral bodies from L3 to the sacrum was found. magnetic resonance imaging disclosed a giant cauda equina tumor with multiple cysts. Central ossification revealed by computed tomography and an unusual myelogram made the preoperative diagnosis difficult. RESULTS: The patient underwent incomplete removal of the tumor, decompression of cysts, and spinal reconstruction. The tumor was proved to be a schwannoma. The postoperative course was uneventful and she has been almost free from low back pain for 3 years and 4 months. CONCLUSIONS: Giant schwannoma in the lumbar spine region is usually excised incompletely, because complete removal had the risk of sacrificing many nerve roots. In spite of the incomplete removal of the tumor, the risk of recurrence is low.
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9/152. Neurosurgical implications of carney complex.

    OBJECT: The authors present their neurosurgical experience with carney complex. carney complex, characterized by spotty skin pigmentation, cardiac myxomas, primary pigmented nodular adrenocortical disease, pituitary tumors, and nerve sheath tumors (NSTs), is a recently described, rare, autosomal-dominant familial syndrome that is relatively unknown to neurosurgeons. neurosurgery is required to treat pituitary adenomas and a rare NST, the psammomatous melanotic schwannoma (PMS), in patients with carney complex. Cushing's syndrome, a common component of the complex, is caused by primary pigmented nodular adrenocortical disease and is not secondary to an adrenocorticotropic hormone-secreting pituitary adenoma. methods: The authors reviewed 14 cases of carney complex, five from the literature and nine from their own experience. Of the 14 pituitary adenomas recognized in association with carney complex, 12 developed growth hormone (GH) hypersecretion (producing gigantism in two patients and acromegaly in 10), and results of immunohistochemical studies in one of the other two were positive for GH. The association of PMSs with carney complex was established in 1990. Of the reported tumors, 28% were associated with spinal nerve sheaths. The spinal tumors occurred in adults (mean age 32 years, range 18-49 years) who presented with pain and radiculopathy. These NSTs may be malignant (10%) and, as with the cardiac myxomas, are associated with significant rates of morbidity and mortality. CONCLUSIONS: Because of the surgical comorbidity associated with cardiac myxoma and/or Cushing's syndrome, recognition of carney complex has important implications for perisurgical patient management and family screening. Study of the genetics of carney complex and of the biological abnormalities associated with the tumors may provide insight into the general pathobiological abnormalities associated with the tumors may provide insight into the general pathobiological features of pituitary adenomas and NSTs.
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10/152. Neurinomas of the brachial plexus: case report.

    Neurinomas, also referred to as neurilemmomas and schwannomas, are rare benign tumours of the peripheral nerves, a low proportion of which arise from the brachial plexus. Authors report a case of an ancient schwannoma arising from the brachial plexus. The tumour, usually asymptomatic, may cause sensory radicular symptoms, or rarely motor deficits in the involved arm. Enucleation of the tumour from the nerve without damage to any of the fascicles is the correct treatment.
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