Cases reported "Hemangioblastoma"

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21/173. Supratentorial haemangioblastoma: appearances on MR imaging.

    Haemangioblastoma is a rare, benign tumour of vascular origin which usually occurs in the posterior fossa. Supratentorial haemangioblastomas are exceptionally rare. In this report we present three cases of supratentorial haemangioblastoma with MRI findings. ( info)

22/173. Multiple cerebellar haemangioblastomas symptomatic during pregnancy.

    A patient with von hippel-lindau disease presented during her seventh week of pregnancy with obstructive hydrocephalus and brainstem compression caused by multiple cerebellar haemangioblastomas. Surgery was performed to relieve the hydrocephalus and remove the right cerebellar tumour. The patient's subsequent course supports the hypothesis of vascular engorgement of cerebellar haemangioblastomas during pregnancy. ( info)

23/173. central nervous system complications of von hippel-lindau disease and pregnancy; perinatal considerations: case report and literature review.

    A 30-year-old woman with von hippel-lindau disease presented at 30 weeks' gestation with a symptomatic cerebellar hemangioblastoma. She underwent a craniotomy for complete removal of the tumor. The postoperative period and the remaining of the pregnancy were uneventful. She delivered under epidural anesthesia after induction for postterm. ( info)

24/173. Haemangioblastoma of a cervical sensory nerve root in Von Hippel-Lindau syndrome.

    Spinal haemangioblastomas are rare, accounting for only about 7% of all central nervous system cases. The case of a 40-year-old woman with a haemangioblastoma arising solely from a cervical sensory nerve root is presented. At operation via a cervical laminectomy, it was possible to resect the tumour en masse with the sensory ramus, by extending the laminectomy through the exit foramen for C6. Haemangioblastomas are commonly intramedullary, and have only been reported in this location on one previous occasion. The patient has Von Hippel-Lindau syndrome and a history of multiple solid tumours. The possible role of the Von Hippel-Lindau tumour suppressor gene in the pathogenesis of these neoplasms is discussed. ( info)

25/173. Clinics in diagnostic imaging (49). Cerebello-tonsillar haemangioblastoma.

    A 39-year-old man with cerebello-tonsillar haemangioblastoma is presented,with emphasis on its morphological and imaging features. Although both computed tomography and cerebral angiography are very useful imaging modalities in helping to establish a radiological diagnosis, magnetic resonance (MR) imaging is more accurate and sensitive in depicting the lesion nature and extent. MR imaging is especially reliable in detecting multiple and other associated lesions within the central nervous system, such that a conclusive diagnosis of the von Hippel-Lindau syndrome can be made in selected cases. It is important to identify isolated haemangioblastomas as they constitute a relatively high percentage of benign resectable tumours within the posterior fossa.The differential diagnosis and imaging features of other posterior fossa tumours are also discussed. ( info)

26/173. 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. ( info)

27/173. Cryptic von hippel-lindau disease: germline mutations in patients with haemangioblastoma only.

    OBJECTIVES: central nervous system haemangioblastoma (HAB) is a major feature of von Hippel-Lindau (VHL) disease, and it is estimated that about 30% of HAB patients have VHL disease. Consequently, it is widely recommended that sporadic HAB patients are screened for clinical and radiological features of VHL disease because of the risk of multiple tumours. We investigated the frequency of VHL germline mutations in patients with HAB only with no clinical or radiological evidence of VHL disease to define the role of molecular genetic analysis in the management of such patients. methods: Eighty four patients with a single HAB (23 Dutch, 61 UK) and four with multiple HAB (two Dutch, two UK) were studied by direct sequencing of the coding region and quantitative Southern blotting. RESULTS: A VHL germline mutation was found in three of 69 (4.3%) single HAB patients aged 50 years or less (three of 84 (3.6%) total single HAB patients). A germline VHL mutation was detected in a 44 year old woman with a solitary cerebellar HAB, as well as in four clinically unaffected close relatives, and in two single HAB cases presenting at the ages of 29 and 36 years. Germline VHL mutations were detected in two of four cases with multiple HAB. CONCLUSIONS: Early detection of VHL disease is important to reduce morbidity and mortality and therefore we recommend that, in addition to conventional clinical and radiological investigations, VHL gene mutation analysis should be offered to all HAB patients younger than 50 years. HAB patients aged >50 years will have a lower a priori risk of VHL disease and further data are required to evaluate the role of routine molecular genetic investigations in late onset HAB cases. The failure to detect germline VHL mutations in some patients with multiple HAB may indicate the presence of somatic mosaicism or additional HAB susceptibility genes. ( info)

28/173. Preoperative embolization of upper cervical cord hemangioblastoma concomitant with venous congestion--case report.

    A 16-year-old male presented with a large, solid hemangioblastoma located in the upper cervical cord manifesting as hyperactive reflexes, subtle weakness, and diminished position sense in all extremities. neuroimaging studies indicated venous congestion due to arteriovenous shunt through the tumor. Preoperative embolization was accomplished without morbidity, and resulted in marked devascularization of the tumor and elimination of an early filling vein. Four days after embolization, the tumor was totally excised without excessive intraoperative bleeding. His neurological deficits gradually improved after surgery. Preoperative embolization is a valuable adjunct to surgical excision of large intramedullary hemangioblastomas, especially those associated with arteriovenous shunt, as cord dysfunction related to venous congestion and the risk of torrential intraoperative bleeding are reduced. ( info)

29/173. Supratentorial hemangioblastoma in a patient with breast cancer. A case report.

    patients with breast cancer may develop cerebral metastasis. radio- and chemotherapy are advocated as an alternative to surgery in such patients. A woman operated on for breast cancer 2 years earlier developed a cerebral lesion. A definite preoperative diagnosis of the lesion was not possible on the basis of CT and MRI findings. The lesion proved to be a supratentorial hemangioblastoma. Neurosurgical treatment is recommended for patients with breast cancer who present a cerebral lesion, since a correct diagnosis may only be possible in the operating theater. ( info)

30/173. 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. ( info)
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