1/55. 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 = 1keywords = spinal (Clic here for more details about this article) |
2/55. Multiple spinal "miliary" hemangioblastomas in von Hippel-Lindau (vHL) disease without cerebellar involvement. A case report and review of the literature.We report on a 57-year-old male presenting with radicular pain in the nerve roots of L5 and S1 on the right side and dysuria. magnetic resonance imaging (MRI) of the lumbar spine showed multiple (up to 20) small, intradural enhancing nodules attached to the cauda equina down to the sacrum, the largest 1 cm in diameter at the level Th12/L1 compressing the conus. Additionally, small nodules in the cervico-thoracal region adjacent to the cord, but no cerebellar or cerebral abnormalities, were detected in a consecutive MRI of the remaining neuroaxis. The histology of a resected lesion at Th12/L1 revealed hemangioblastoma of the reticular type. Together with a history of left eye enucleation performed 17 years ago for angiomatosis of the retina and the immunohistochemical detection of von Hippel-Lindau (vHL) protein within the removed spinal hemangioblastoma, a diagnosis of vHL disease was established. family history and screening for visceral manifestations of vHL disease were negative. In contrast to cerebellar or solitary spinal hemangioblastomas, multiple spinal hemangioblastomas without cerebellar involvement in vHL represent unusual manifestations. Unlike the case for solitary lesions in non-syndromic patients, a surgical cure does not seem feasible in this case. The role of treatment modalities is discussed.- - - - - - - - - - ranking = 1.1666666666667keywords = spinal (Clic here for more details about this article) |
3/55. 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 = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
4/55. Retroperitoneal peripheral hemangioblastoma: a case report and review of the literature.central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord. Peripheral involvement is rare; only isolated case reports have been identified. We report an unusual case of hemangioblastoma involving the retroperitoneum. A 47-year-old African-American man presented with polycythemia on routine laboratory testing. Computed tomography revealed a large retroperitoneal mass near the pancreas, in a left suprarenal location, without adrenal involvement and without attachment to a nerve. Although hemangioblastoma may be associated with the von Hippel-Lindau syndrome, this patient did not have any of the stigmata of this disease. The histologic features included a highly vascular tumor with cellular areas composed of plump, pleomorphic spindled and epithelioid (stromal) cells with variable cytoplasmic lipid vacuoles and hypocellular areas with inflammatory cells and collagenous fibrils. Immunohistochemical staining showed that the tumor (stromal) cells were positive for vimentin, calponin, S-100 protein, neuron-specific enolase, and CD57 and negative for glial fibrillary acidic protein, cytokeratins, epithelial membrane antigen, CD34, HMB-45, desmin, and the actins. These morphologic and immunohistochemical findings are consistent with hemangioblastoma. To our knowledge this is the first reported case of a hemangioblastoma in this location. Based on this case we conclude that hemangioblastoma may occur in the retroperitoneum and outside of the central nervous system in a patient without von Hippel-Lindau syndrome. The immunoprofile of this case suggests that hemangioblastomas are mesenchymal neoplasms exhibiting both neural and myofibroblastic differentiation.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
5/55. Resection of suprasellar tumors by using a modified transsphenoidal approach. Report of four cases.Generally accepted contraindications to using a transsphenoidal approach for resection of tumors that arise in or extend into the suprasellar region include a normal-sized sella turcica, normal pituitary function, and adherence of tumor to vital intracranial structures. Thus, the transsphenoidal approach has traditionally been restricted to the removal of tumors involving the pituitary fossa and, occasionally, to suprasellar extensions of such tumors if the sella is enlarged. However, conventional transcranial approaches to the suprasellar region require significant brain retraction and offer limited visualization of contralateral tumor extension and the interface between the tumor and adjacent structures, such as the hypothalamus, third ventricle, optic apparatus, and major arteries. In this paper the authors describe successful removal of suprasellar tumors by using a modified transsphenoidal approach that circumvents some of the traditional contraindications to transsphenoidal surgery, while avoiding some of the disadvantages of transcranial surgery. Four patients harbored tumors (two craniopharyngiomas and two hemangioblastomas) that arose in the suprasellar region and were located either entirely (three patients) or primarily (one patient) within the suprasellar space. All patients had a normal-sized sella turcica. Preoperatively, three of the four patients had significant endocrinological deficits signifying involvement of the hypothalamus, pituitary stalk, or pituitary gland. Two patients exhibited preoperative visual field defects. For tumor excision, a recently described modification of the traditional transsphenoidal approach was used. Using this modification, one removes the posterior portion of the planum sphenoidale, allowing access to the suprasellar region. Total resection of tumor was achieved (including absence of residual tumor on follow-up imaging) in three of the four patients. In the remaining patient, total removal was not possible because of adherence of tumor to the hypothalamus and midbrain. One postoperative cerebrospinal fluid leak occurred. Postoperative endocrinological function was worse than preoperative function in one patient. No other new postoperative endocrinological or neurological deficits were encountered. This study demonstrates the feasibility of using a modified transsphenoidal approach for resection of certain suprasellar, nonpituitary tumors.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
6/55. Extramedullary hemangioblastoma of the conus medullaris.BACKGROUND: We report the case of an extramedullary pathologically proven hemangioblastoma of the conus medullaris. As spinal dural arteriovenous fistulas most commonly present with a conus medullaris syndrome, our presentation of the MRI, myelographic, and angiographic findings of this unique lesion may be useful in differentiating these two entities. CLINICAL MATERIAL: We report the case of a 57 year old woman with a two year history of progressive low back and right lower extremity pain and weakness. Spinal MRI and myelography demonstrated serpiginous vasculature on the dorsum of the spinal cord consistent with either a vascular tumor or malformation. Selective spinal angiography was thus undertaken by the neuroendovascular team which revealed a tumor nodule consistent with vascular tumor. T12-L1 laminectomy was performed and a 6 mm vascularized tumor was found in the intradural extramedullary compartment adjacent to the conus medullaris. The tumor was completely removed and pathological analysis was consistent with hemangioblastoma. CONCLUSION: This report documents a unique location for extramedullary spinal hemangioblastomas. Although both MRI and myelography are helpful in studying these lesions, angiography remains the gold standard in differentiating between vascular tumor and malformation. We suggest that the angiography be performed by a neurointerventional team to facilitate embolization, should this be warranted.- - - - - - - - - - ranking = 0.66666666666667keywords = spinal (Clic here for more details about this article) |
7/55. Recurrent (nonfamilial) hemangioblastomas involving spinal nerve roots: case report.OBJECTIVE AND IMPORTANCE: Spinal nerve root hemangioblastomas are rare and are reported mainly in patients with von Hippel-Lindau (VHL) syndrome. The pathogenesis of so-called nonfamilial lesions is virtually unknown. We discuss, mainly from a molecular perspective, a unique patient with sporadic, recurrent hemangioblastomas restricted to spinal nerve roots. CLINICAL PRESENTATION: A 53-year-old man who had had a surgically corrected lumbosacral meningomyelocele presented on at least three occasions during a 17-year period with multifocal capillary hemangioblastomas involving spinal nerve roots. On each occasion, tumors appeared on a different nerve root, with the majority located in the midcervical segments. The patient had no clinical features or family history of VHL syndrome. TECHNIQUE: To obtain a clearer understanding of the pathogenesis of this unusual case and its relationship to VHL syndrome, molecular analysis of the VHL gene was performed by use of complete sequence analysis and loss of heterozygosity studies on deoxyribonucleic acid derived from the patient's blood leukocytes and three separately resected hemangioblastomas. CONCLUSION: Germ-line molecular analysis performed on all three exons in the VHL gene coding region did not indicate that any mutations were present. loss of heterozygosity analysis of deoxyribonucleic acid from the three hemangioblastoma resections showed normal heterozygosity in the 3p25-26 region. Complete VHL gene sequence analysis did not demonstrate a somatic mutation in the coding region of the VHL gene in any of the three tumors, thereby supporting the loss of heterozygosity data that a molecular event directly involving the VHL gene may not be the causative factor in their tumorigenesis.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
8/55. hemangioblastoma arising in the skin.Hemangioblastomas are intracranial and intraspinal tumors arising sporadically or in patients with von hippel-lindau disease. To date, hemangioblastomas have not been described in the skin. Proliferating clear cells with a variable vascular component are found histologically. These clear cells stain for neuron-specific enolase but not cytokeratin or epithelial membrane antigen, allowing them to be differentiated from metastatic renal cell carcinoma.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
9/55. Metastasis of renal cell carcinoma to central nervous system hemangioblastoma in two patients with von hippel-lindau disease.Here we report tumor-to-tumor metastases identified in two patients with von Hippel-Lindau (VHL) disease. The first patient had bilateral renal carcinomas and multiple cerebellar hemangioblastomas, and the second patient had a renal carcinoma and multiple hemangioblastomas in the retina, cerebellum and spinal cord. A cerebellar lesion from the first patient and a spinal lesion from the second patient contained two distinct components. The inner part of these tumors consisted of a nested mass of polygonal clear cells that expressed cytokeratin and epithelial membrane antigen, while the outer part of the tumors showed proliferation of capillaries and intervening foamy stromal cells that were negative for cytokeratin and epithelial membrane antigen. The tumors were thus considered to be hemangioblastomas complicated by metastatic lesions of renal cell carcinoma of clear cell type. These cases indicate that tumor-to-tumor metastasis should be considered when hemangioblastoma contains a clear cell carcinoma component in the setting of VHL disease, and that immunohistochemical staining for cytokeratin and epithelial membrane antigen is useful for the diagnosis.- - - - - - - - - - ranking = 0.33333333333333keywords = spinal (Clic here for more details about this article) |
10/55. hemangioblastoma causing cervical neural foraminal widening.We present the MRI findings in a case of a 24-year-old woman with spinal hemangioblastoma, causing neural foraminal widening by producing a dumbbell mass in the lower cervical region. Hemangioblastomas can very rarely present as an intradural extramedullary lesions and this case is another exceptional pathology which should be considered among the differential diagnosis of enlarged intervertebral foramen due to neoplastic processes.- - - - - - - - - - ranking = 0.16666666666667keywords = spinal (Clic here for more details about this article) |
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