1/42. Multiple spinal meningiomas--a case report.Multiple spinal meningiomas are relatively rare and account for only 2 to 3.5% of all spinal meningiomas. A case of multiple meningiomas of the thoracic (T) spine was reported. This 77-year-old woman was admitted with progressive motor weakness and sensation disturbance in both legs for 4 months. Neurological findings on admission revealed paraparesis, hyperesthesia, and hypalgesia below the dermatome of T10 level and hyporeflexia of both ankles. Urinary and stool incontinence were also noted. magnetic resonance imaging (MRI) documented two intradural extramedullary lesions with different intensity at the T9 and T12 levels. Both tumors were totally resected. Histopathologic examination showed all psammomatous meningiomas with more or less psammoma bodies. There were no any manifestations of von Recklinghausen's disease in this patient. MRI is a choice of diagnostic tool to detect spinal tumors, especially multiple spinal meningiomas. Different intensities in MRI may infer different components of meningiomas. Follow-up study after operation three months later disclosed she could walk very well.- - - - - - - - - - ranking = 1keywords = von (Clic here for more details about this article) |
2/42. Extradural spinal hemangioblastomas: report of two cases.Two cases of predominantly extraspinally extra- and intradural spinal cord hemangioblastomas in two patients each with and without von Hippel-Lindau-disease are reported. Preoperative MRI and angiographic findings are presented and the differential diagnosis is discussed. The surgical procedure is described and the literature of hemangioblastomas in this rare localization is reviewed. Improvements in both radiologic diagnosis and microsurgical techniques, and consequent screening programs will enhance life expectancy in patients afflicted with von hippel-lindau disease.- - - - - - - - - - ranking = 2keywords = von (Clic here for more details about this article) |
3/42. dna-based diagnosis of the von Hippel-Lindau syndrome.PURPOSE: To evaluate the etiology of a unilateral hemangioblastoma noted in a male with a family history remarkable only for spine surgery in the proband's father. methods: Genomic dna was isolated from peripheral blood of family members, and the three exons of the von Hippel-Lindau gene were examined for mutations by direct sequencing. RESULTS: A three base pair (bp) deletion in exon 1 of the VHL gene was found in the father and both sons. This in-frame deletion results in the loss of a phenylalanine residue from the von Hippel-Lindau protein product, at amino acid position 76. CONCLUSION: Genetic screening has confirmed that von Hippel-Lindau syndrome is responsible for the hemangioblastoma in the proband. magnetic resonance imaging scans performed as a consequence of these results indicated spinal tumors present in the father and tumors present in the cerebellum of the proband's sibling. As close, lifelong follow-up is warranted with this disease, this case demonstrates the value of dna testing in patients with ocular findings consistent with von hippel-lindau disease in the absence of a recognized family history.- - - - - - - - - - ranking = 8keywords = von (Clic here for more details about this article) |
4/42. Intramedullary schwannoma of the spinal cord. A case report and review of the literature.A 9-year-old boy presented with the numbness in both arms and hands, and neck stiffness. On examination, he had a slight quadriparesis and restricted neck movements. There were no signs of von Recklinghausen's disease. magnetic resonance imaging (MRI) scan disclosed a gadolinium enhanced intramedullary tumor located at C6-T1 associated with syringomyelia. C6-T1 laminectomies were performed and the intramedullary tumor was totally removed by a microsurgical technique. Postoperative course was uneventful. The pathological examination revealed an intramedullary schwannoma. The occurrence of intramedullary schwannoma in a patient without signs of von Recklinghausen's disease is extremely rare. We have been able to find 57 cases of intramedullary schwannoma reported in the literature. Intramedullary schwannomas are usually seen in males. The ages of the patients ranged from 9 to 75 years (mean 40.44 years). Only 4 cases in the pediatric age group have been reported. The duration of symptoms ranged from 3 months to 20 years (mean 31.03 months). Symptoms and signs varied with the location of tumor. The vertebral levels of intramedullary schwannomas were usually cervical (61%). MRI has been the choice of diagnostic tool in the cases reported since 1986. The majority of the cases showed either a partial or complete recovery in the postoperative period.- - - - - - - - - - ranking = 2keywords = von (Clic here for more details about this article) |
5/42. Malignant pheochromocytoma with progressive paraparesis in von hippel-lindau disease.Pheochromocytomas are a feature of the von hippel-lindau disease spectrum, a multisystem disorder of autosomal dominant inheritance. Pheochromocytomas are, however, observed during life with a lower frequency than other features of this disease, such as retinal angiomas, haemangioblastomas of the CNS, and renal carcinomas. We present the highly unusual case of a patient who required an emergency operation for an intradural extramedullary thoracic tumour which was clinically suggestive initially of neurinoma. We present evidence from NMR, histological and isotope scan investigations of this being a pheochromocytoma metastasis and of an additional right-sided paraganglioma at the same height. A detailed history revealed that this patient had suffered from four other pheochromocytomas and two other paragangliomas, in addition to retinal angiomatosis of von hippel-lindau disease. This case is extraordinary due to (i) the unusual site of the metastasis, (ii) the neurological requirement for an emergency operation of pheochromocytoma, (iii) metastasis of pheochromocytoma in von hippel-lindau disease (only eight previous cases), and (iv) the number of recurrent pheochromocytomas. It clearly demonstrates the necessity for frequent and life-long follow-up in von hippel-lindau disease.- - - - - - - - - - ranking = 8keywords = von (Clic here for more details about this article) |
6/42. Intramedullary spinal cord metastasis as a first manifestation of a renal cell carcinoma: report of a case and review of the literature.The authors report the case of a 70-year-old woman who developed a Brown-Sequard-syndrome within 6 weeks caused by an intramedullary spinal cord metastasis of an occult renal cell carcinoma. Intramedullary metastases are rare and represent only 4-8.5% of central nervous system metastases. An important feature of intramedullary metastases is the rapid progression of neurological deficits which necessitates rapid treatment. There are only eight earlier reports of intramedullary metastasis due to renal cell carcinoma (Schiff D, O'Neill BP. Intramedullary spinal cord metastases: clinical features and treatment outcome. neurology 1996;47:906-12; Belz P. Ein Fall von intramedullaerer Grawitz-Metastase im Lumbalmark. Frankfurt Z Pathol 1912;10:431-44; Gaylor JB, Howie JW. Brown-Sequard-syndrome. A case of unusual aetiology. J Neurol Neurosurg psychiatry 1938;1:301-5; Kawakami Y, Mair WGP. Haematomyelia due to secondary renal carcinoma. Acta Neuro Pathol 1973;26:85-92; Strang RR. Metastatic tumor of the cervical spinal cord. Med J Aust 1962;1:205-6; Von Pfungen. Uber einige Falle von Haematomyelie nichttraumatischen Ursprungs. Wien Klin Rdsch 1906;20:44-50; Weitzner S. Coexistent intramedullary metastasis and syringomyelia of cervical spinal cord. Report of a case. neurology 1960;674-8). To the best of our knowledge this is the first report on a patient in whom symptoms from the metastasis of a renal cell carcinoma preceded the detection of the primary tumor. This report presents the clinical, neuroradiological and histopathological findings of an intramedullary metastasis of a renal cell carcinoma and provides an overview of the literature on intramedullary spinal cord metastases.- - - - - - - - - - ranking = 2keywords = von (Clic here for more details about this article) |
7/42. Clinical and molecular analysis of disseminated hemangioblastomatosis of the central nervous system in patients without von hippel-lindau disease. Report of four cases.Hemangioblastomas of the central nervous system (CNS) may occur sporadically or in association with von Hippel-Lindau (VHL) syndrome. The authors present four patients with no family history or clinical evidence of VHL syndrome in whom extensive, progressive, en plaque coating of the brainstem and spinal cord with hemangioblastomas developed 1 to 8 years after complete resection of a solitary cerebellar hemangioblastoma. Analysis included detailed physical, biochemical, radiological, and pathological examinations in all four patients, combined with family pedigree analysis. In addition, a detailed investigation of the VHL gene was undertaken. Allelic loss, comparative genomic hybridization (CGH), single-stranded conformational polymorphism screening, CpG island methylation status, and x chromosome inactivation clonality analyses were performed. Although there was no evidence of germline alterations in the VHL gene on clinical and radiological examination or in the family history (all four patients) or analysis of peripheral blood (three patients), somatic deletion of one copy of the VHL gene occurred in these tumors. These findings indicate that the multiple, separate deposits of tumors were likely derived from a single clone. Results of CGH indicate that one or several additional genes are probably involved in the malignant behavior of the hemangioblastomas in these patients. Furthermore, the malignant biological and clinical behavior of these tumors, in which multiple sites of subarachnoid dissemination developed 1 to 8 years after initial complete resection, followed by progressive tumor growth and death of the patients, occurred despite a histological appearance typical of benign hemangioblastomas. Malignant hemangioblastomatosis developed 1 to 8 years after resection of an isolated cerebellar hemangioblastoma. Alterations of the VHL gene may be permissive in this setting, but other genes are likely to be the source of the novel biological and clinical presentation of the disseminated hemangioblastomas in these patients. This appears to represent a novel condition in which the product of one or more mutations in several genes permits malignant tumor behavior despite retention of a benign histological picture, a circumstance previously not recognized in CNS tumors.- - - - - - - - - - ranking = 5keywords = von (Clic here for more details about this article) |
8/42. Surgical management of spinal cord hemangioblastomas in patients with von hippel-lindau disease.OBJECT: Von Hippel-Lindau (VHL) disease is an autosomal-dominant disorder frequently associated with hemangioblastomas of the spinal cord. Because of the slow progression, protean nature, and high frequency of multiple spinal hemangioblastomas associated with VHL disease, the surgical management of these lesions is complex. Because prior reports have not identified the factors that predict which patients with spinal cord hemangioblastomas need surgery or what outcomes of this procedure should be expected, the authors have reviewed a series of patients with VHL disease who underwent resection of spinal hemangioblastomas at a single institution to identify features that might guide surgical management of these patients. methods: Forty-four consecutive patients with VHL disease (26 men and 18 women) who underwent 55 operations with resection of 86 spinal cord hemangioblastomas (mean age at surgery 34 years; range 20-58 years) at the National Institutes of health were included in this study (mean clinical follow up 44 months). Patient examination, review of hospital charts, operative findings, and magnetic resonance imaging studies were used to analyze surgical management and its outcome. To evaluate the clinical course, clinical grades were assigned to patients before and after surgery. Preoperative neurological status, tumor size, and tumor location were predictive of postoperative outcome. patients with no or minimal preoperative neurological dysfunction, with lesions smaller than 500 mm3, and with dorsal lesions were more likely to have no or minimal neurological impairment. Syrinx resolution was the result of tumor removal and was not influenced by whether the syrinx cavity was entered. CONCLUSIONS: Spinal cord hemangioblastomas can be safely removed in the majority of patients with VHL disease. Generally in these patients, hemangioblastomas of the spinal cord should be removed when they produce symptoms or signs.- - - - - - - - - - ranking = 4keywords = von (Clic here for more details about this article) |
9/42. Comparison of anterior and posterior surgical approaches in the treatment of ventral spinal hemangioblastomas in patients with von hippel-lindau disease.OBJECT: Von Hippel-Lindau (VHL) disease is an autosomal-dominant neoplastic syndrome with manifestations in multiple organs, which is evoked by the deletion or mutation of a tumor suppressor gene on chromosome 3p25. Spinal hemangioblastomas (40% of VHL disease-associated lesions of the central nervous system) arise predominantly in the posterior aspect of the spinal cord and are often associated with an intraspinal cyst. Rarely, the tumor develops in the anterior aspect of the spinal cord. Ventral spinal hemangioblastomas are a surgical challenge because of difficult access and because vessels feeding the tumor originate from the anterior spinal artery. The goal of this study was to clarify whether an anterior or posterior surgical approach is better for management of hemangioblastomas of the ventral spinal cord. METHOD:. The authors performed a retrospective analysis of clinical outcomes and findings on magnetic resonance (MR) imaging studies in eight patients (two women and six men with a mean age of 34 /- 15 years) who underwent resection of ventral spinal hemangioblastomas (nine tumors: five cervical and four thoracic). Two surgical approaches were used to resect these tumors. A posterior approach was selected to treat five patients (laminectomy and posterior myelotomy in four patients and the posterolateral approach in one patient); an anterior approach (corpectomy and arthrodesis) was selected to treat the remaining three patients. Immediately after surgery, the ability to ambulate remained unchanged in patients in whom an anterior approach had been performed, but deteriorated significantly in patients in whom a posterior approach had been used, because of motor weakness (four of five patients) and/or proprioceptive sensory loss (three of five patients). This difference in ambulation, despite significant improvements over time among patients in the posterior access group, remained significant 6 months after surgery. In all cases, MR images revealed complete resection of the tumor and in five patients significant or complete resolution of the intramedullary cyst was demonstrated (present in six of eight patients). CONCLUSIONS: The outcomes of these eight patients with hemangioblastomas of the ventral spinal cord indicate that both immediate and long-term results are better when an anterior approach is selected for resection.- - - - - - - - - - ranking = 4keywords = von (Clic here for more details about this article) |
10/42. Metastasis of renal cell carcinoma to haemangioblastoma of the spinal cord in von hippel-lindau disease: case report and review of the literature.A case is presented demonstrating the unusual phenomenon of a renal cell carcinoma metastasising to a spinal haemangioblastoma in a patient with von Hippel-Lindau (VHL) disease. Recent advances in the molecular biology of VHL disease relevant to possible mechanisms of tumour-to-tumour metastasis are briefly reviewed.- - - - - - - - - - ranking = 5keywords = von (Clic here for more details about this article) |
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