Cases reported "Nervous System Neoplasms"

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1/21. Neuroendocrine carcinoma of the stomach with extensive somatostatin immunoreactivity.

    upper gastrointestinal tract neuroendocrine tumors producing predominantly somatostatin have thus far been described only in the duodenum; their characteristic features include the frequent presence of psammoma bodies (psammomatous somatostinomas), and the association with von Recklinghausen's neurofibromatosis. Gastric neuroendocrine tumors, on the other hand, tend to display immunoreactivity to serotonin but may include small subpopulations producing gastrin, motilin, pancreatic polypeptide, and somatostatin. In this report we describe a neuroendocrine carcinoma of the stomach with rapidly fatal outcome, displaying neurosecretory granules by electron microscopy and immunoreactivity to pan-neuroendocrine markers, ie, chromogranin and neuron-specific enolase. The only neuroendocrine regulatory peptide detected in the tumor was somatostatin, identified by immunohistochemistry in the majority of neoplastic cells. In contrast with duodenal somatostinomas, there were no psammoma bodies and no demonstrable association with von Recklinghausen's neurofibromatosis. To our knowledge this appears to be the first report of a malignant neuroendocrine tumor with diffuse somatostatin immunoreactivity.
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ranking = 1
keywords = neurofibroma
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2/21. Neurofibromatosis-associated nerve sheath tumors. Case report and review of the literature.

    In this paper the authors describe a patient with neurofibromatosis Type 1 (NF1) who presented with sequelae of this disease. They also review the current literature on NF1 and NF2 published between 2001 and 2005. The method used to obtain information for the case report consisted of a family member interview and a review of the patient's chart. For the literature review the authors used the search engine Ovid medline to identify papers published on the topic between 2001 and 2005. Neurofibromatosis Type 1 appears in approximately one in 2500 to 4000 births, is caused by a defect on 17q11.2, and results in neurofibromin inactivation. The authors reviewed the current literature with regard to the following aspects of this disease: 1) diagnostic criteria for NF1; 2) criteria for other NF1-associated manifestations; 3) malignant peripheral nerve sheath tumors (PNSTs); 4) the examination protocol for a patient with an NF1-related NST; 5) imaging findings in patients with NF1; 6) other diagnostic studies; 7) surgical and adjuvant treatment for NSTs and malignant PNSTs; and 8) hormone receptors in NF1-related tumors. Pertinent illustrations are included. Neurofibromatosis Type 2 occurs much less frequently than NF1, that is, in one in 33,000 births. Mutations in NF2 occur on 22q12 and result in inactivation of the tumor suppressor merlin. The following data on this disease are presented: 1) diagnostic criteria for NF2; 2) criteria for other NF2 manifestations; 3) malignant PNSTs in patients with NF2; 4) examination protocol for the patient with NF2 who has an NST; and 5) imaging findings in patients with NF2. Relevant illustrations are included. It is important that neurosurgeons be aware of the sequelae of NF1 and NF2, because they may be called on to treat these conditions.
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ranking = 0.5
keywords = neurofibroma
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3/21. Video-assisted thoracoscopic dissection of the brachial plexus: cadaveric study and illustrative case.

    OBJECTIVE: Standard surgical approaches to the brachial plexus require an open operative technique with extensive soft tissue dissection. A transthoracic endoscopic approach using video-assisted thoracoscopic surgery (VATS) was studied as an alternative direct operative corridor to the proximal inferior brachial plexus. methods: VATS was used in cadaveric dissections to study the anatomic details of the brachial plexus at the thoracic apex. After placement of standard thoracoscopic ports, the thoracic apex was systematically dissected. The limitations of the VATS approach were defined before and after removal of the first rib. The technique was applied in a 22-year-old man with neurofibromatosis who presented with a large neurofibroma of the left T1 nerve root. RESULTS: The cadaveric study demonstrated that VATS allowed for a direct cephalad approach to the inferior brachial plexus. The C8 and T1 nerve roots as well as the lower trunk of the brachial plexus were safely identified and dissected. Removal of the first rib provided exposure of the entire lower trunk and proximal divisions. After the fundamental steps to the dissection were identified, the patient underwent a successful gross total resection of a left T1 neurofibroma with VATS. CONCLUSION: VATS provided an alternative surgical corridor to the proximal inferior brachial plexus and obviated the need for the extensive soft tissue dissection associated with the anterior supraclavicular and posterior subscapular approaches.
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ranking = 1.5
keywords = neurofibroma
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4/21. Glioneurofibroma: renaming the pediatric "gliofibroma": a neoplasm composed of schwann cells and astrocytes.

    Three children had central nervous system tumors with histologic and ultrastructural features corresponding to those of tumors previously described as "gliofibromas." These features, which include a composite appearance with glial and mesenchymal elements, with glial fibrillary acidic protein (GFAP)-containing and GFAP-immunonegative cells, diffuse S-100 immunoreactivity, and basal lamina wrapping processes of both cell types, suggest that the "mesenchymal" cells are schwann cells, not fibroblasts. We therefore propose to rename this entity "glioneurofibroma." The clinical behavior of these lesions is uncertain but is more often indolent or benign.
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ranking = 2.5
keywords = neurofibroma
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5/21. Intraspinal neurothekeoma (nerve sheath myxoma). A report of two cases.

    Neurothekeomas (nerve sheath myxomas) are rare benign cutaneous tumors. The authors describe two spinal intradural cases that show histologic, immunohistochemical, and electron microscopic features identical to their cutaneous counterparts. The authors' findings suggest histogenesis from Schwann's cells or undifferentiated nerve sheath precursor cells. Neurothekeomas in unusual locations should be differentiated from myxoid neurofibromas, perineuriomas, and soft tissue myxomas.
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ranking = 0.5
keywords = neurofibroma
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6/21. Occurrence of both neurofibromatoses 1 and 2 in the same individual with a rapidly progressive course.

    We describe a family in which the father had neurofibromatosis-1 and the mother neurofibromatosis-2. Their son presented at the age of 8 years with bilateral acoustic neuromas, meningioma, and numerous neurofibromas. We believe that the occurrence of the genes responsible for both forms of neurofibromatosis in the same patient had a synergistic effect on the early rapid growth of neurofibromatoses 1 and 2 neoplasms.
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ranking = 4.5
keywords = neurofibroma
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7/21. Schwannomatosis. An unusual variant of neurofibromatosis or a distinct clinical entity?

    Multiple schwannomas have frequently been seen in patients with neurofibromatosis. Recently, the association of multiple cutaneous schwannomas, central nervous system tumors, and various neurologic deficits has been described in Japanese patients as a condition called schwannomatosis. We describe the first non-Japanese cases of schwannomatosis and compare and contrast this unusual condition with the well-known variants of neurofibromatosis. We conclude that the features of schwannomatosis are distinct and define a condition that does not fit into the current classification scheme of neurofibromatosis. The occurrence of multiple cutaneous schwannomas in the absence of cardinal features of neurofibromatosis may indicate the presence of central nervous system tumors or various neurologic deficits.
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ranking = 4
keywords = neurofibroma
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8/21. Two epithelioid malignant schwannomas in a patient with neurofibromatosis. Cytology, histology and dna-flow-cytometry.

    Cytological, histological and dna-ploidy findings of 2 epithelioid malignant schwannomas arising in a patient with von Recklinghausen's neurofibromatosis are described. In both primary tumors, i.e. in the thigh and in the thoracic wall, origin from a neurofibromatous nerve could be established. Within 2 years after the manifestation of the first malignant tumor the patient died of widely metastasized disease. dna-flow cytometry of several neurofibromas revealed diploid stemlines, but both malignant primary tumors and a lung metastasis appeared to be aneuploid. Presumably, the primary tumors also contained cell populations with a diploid stem line.
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ranking = 3.5
keywords = neurofibroma
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9/21. "Vascular neurofibromatosis" and infantile gangrene.

    An 11-year-old boy with slowly progressive gangrene caused by vasculopathy similar to that of neurofibromatosis (NF) type 1 (NF I; von Recklinghausen disease [NFvR]) and a newborn girl with idiopathic gangrene with vascular changes resembling those of NFvR prompted the analysis of all 105 propositi with NF (NF I and NF II) evaluated between January 2, 1982, and December 31, 1986, at the genetics clinic of University of South florida. They were analyzed for renal hypertension, symptomatic ischemia, and known vascular changes. One additional 27-month-old boy with NFvR was found to have extensive vascular changes with renal hypertension. The vasculopathy indicated asymmetric over/undergrowth of cellular and extracellular components of the vascular wall and implied dysregulation of the paracrine growth mechanism. Immunocytochemical studies of affected vessels were done only in the 11-year-old boy and showed positive neuron-specific enolase, S-100 protein, and glial fibrillary acidic protein (GFAP) reactions indicative of Schwann cell involvement. The vascular changes in children with NFvR are mostly asymptomatic; however hypertension secondary to renal artery stenosis and/or Moya-moya disease have been reported infrequently. Our patients with vasculopathies provoked thoughts in regard to the so-called vascular NF, its place in current NF nomenclature and classification, relationship to fibromuscular dysplasia (FMD), and possible role in infantile gangrene.
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ranking = 2.5
keywords = neurofibroma
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10/21. Neurofibromatosis and associated neuroectodermal tumors: a congenital neurocristopathy.

    The synchronous occurrence of neurofibromatosis and neuroblastoma has been labeled in the recent literature as a chance event. We report 2 cases of newborn infants with congenital neurofibromatosis and a similar midline pattern of multiple Schwann cell and neuroblastic tumors; other types of ectomesenchymal tumor differentiation are documented, along with supportive ultrastructural and immunohistochemical studies. The tumors may take an aggressive, fatal course despite maximal multimodality antitumor therapy. These 2 cases are reported, with additional literature review, to document a clinically recognizable neurocristopathy that links neuroblastic tumors and neurofibromatosis.
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ranking = 1.5
keywords = neurofibroma
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