Cases reported "Hemangioblastoma"

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1/17. An intracranial aneurysm on the feeding artery of a cerebellar hemangioblastoma. Case report.

    A case of cerebellar hemangioblastoma with a coexistent arterial aneurysm on the feeding artery of the tumor is reported. The patient presented with an acute onset of headache, loss of consciousness, and left-sided hemiparesis due to a posterior fossa hemorrhage found adjacent to a hemangioblastoma. Four-vessel angiography revealed an aneurysm on the anterior inferior cerebellar artery (AICA), which was the main feeding vessel of the hemangioblastoma. Successful total excision of the hemangioblastoma and clipping of the AICA aneurysm achieved in a one-stage operation was demonstrated on postoperative angiography.
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2/17. Tentorial vascularization in solid hemangioblastoma--case report.

    A 40-year-old female was admitted to the hospital with complaints of headache worsening gradually over a 1-month duration. Her past history included surgery to treat a left cerebellar cystic lesion 3 years before, and an untreated small solid right supracerebellar lesion of 1 cm diameter. On admission, magnetic resonance imaging showed that the right cerebellar lesion had grown to approximately 4 cm diameter abutting the tentorium and causing obstructive hydrocephalus. She also had two more small lesions, a right supratentorial solid lesion with cystic component near the splenium and an intramedullary cystic lesion at the C-2 level. Right suboccipital craniectomy was done. The vascular attachments between the superior aspect of the tumor and the tentorium were coagulated and the tumor was totally removed. C1-2 laminectomy was also performed to drain the intramedullary cyst. The patient deteriorated and lost consciousness with respiratory arrest 6 hours postoperatively and was reoperated for intracerebellar hematoma due to oozing from the tentorial vessels. Histological investigation revealed hemangioblastoma. Dural tentorial vascular attachments in solid hemangioblastomas located subjacent to the tentorium may cause early postoperative complications of hematoma at the site of vascular attachment following the resection. Computed tomography study in the early postoperative period is helpful to identify this problem.
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3/17. Isolated hemangioblastoma of the filum terminale. Case report.

    The filum terminale is an exceptional location for isolated hemangioblastoma. Only five cases are reported in literature, prior to the magnetic resonance imaging era. A 57-year-old man was referred to our Department with a prolonged history of progressive back pain, particularly severe when recumbent, and recurrent unilateral sciatalgia. Computed tomography demonstrated a non-homogeneous mass at L4 level. magnetic resonance imaging displayed earliness and homogeneous enhancement of the mass with tortuous vessels above the rostral pole of the tumour, suggesting the diagnosis of a vascular tumour. The tumour was totally removed. Histological examination confirmed the hemangioblastoma diagnosis. The case indicates that hemangioblastoma, although uncommon, must be taken into consideration in cauda equina tumour diagnosis, and also emphasises the specificity of magnetic resonance features.
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4/17. Myelopathy and sciatica induced by an extradural S1 root haemangioblastoma.

    Haemangioblastomas are vascular tumours which mainly involve the central nervous system and retina, often in the setting of von hippel-lindau disease. Haemangioblastomas occurring outside the central nervous system are uncommon. Wherever it is, recognising this tumour prior to surgery is desirable, as preoperative embolisation may be considered. We report the clinical, imaging and pathological features of a sporadic sacral root haemangioblastoma in a 58-year-old man with chronic sciatica and myelopathy. The diagnosis was questioned preoperatively because an enlarged sacral foramen, seen to be filled by a highly vascular, enhancing mass and dilated vessels. Myelopathy was attributed to the presumed high venous pressure resulting from increased flow in veins draining the vascular tumour. Microneurosurgical excision was performed after endovascular embolisation and led to persistent clinical improvement.
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5/17. Atraumatic bloodless removal of intramedullary hemangioblastomas of the spinal cord.

    OBJECT: The goal of this study was to summarize the author's personal experience in the surgical treatment of 19 patients with intramedullary spinal cord hemangioblastomas. methods: All cases were from the author's private practice and were treated between 1967 and 1990. In all cases the intramedullary hemangioblastomas were totally removed by using a unique microsurgical technique of bipolar coagulation, which is fully described in this paper. A bipolar forceps was used to shrink each tumor and detach it from its feeding and draining vessels. Tumor resection was successfully accomplished in all patients. blood loss was minimal, averaging less than 100 ml, and what little bleeding occurred did so during laminotomy or laminectomy. No bleeding occurred during tumor removal, and no transfusions were given. All patients were symptomatic preoperatively, and all recovered or improved following surgery. CONCLUSIONS: The technique described in this paper makes tumor removal safe, effective, and relatively easy.
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6/17. 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.
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7/17. Familial incidence of obstructive hydrocephalus due to posterior fossa tumours leading to the diagnosis of von hippel-lindau disease--a case report.

    von hippel-lindau disease (VHLD) is an autosomal dominant disorder predisposing to the development of different histological types of tumours with predominance of CNS haemangioblastomas in the majority of cases. We present a case of a 35-year-old man with obstructive hydrocephalus due to multiple posterior fossa tumours. Familial history revealed two relatives operated on previously because of a posterior fossa tumour (in one case haemangioblastoma was diagnosed). During surgery two cerebellar tumours were removed radically via the suboccipital approach. On a histopathological examination the typical features of haemangioblastoma were found in both tumours composed of stromal cells and a dense network of vessels, mostly capillaries. The presence of neoplastic infiltration with angiogenesis in the surrounding cerebellar tissue was evidenced in the presented case. Based on the clinical and histopathological data VHLD was diagnosed. Genetic analysis confirmed the presence of the von Hippel-Lindau gene mutation. The case presented suggests that genetic disregulation in VHLD may be responsible for a more aggressive behaviour of VHLD-related haemangioblastomas in comparison with sporadic disease.
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8/17. Leptomeningeal hemangioblastomatosis in a case of von hippel-lindau disease: case report.

    OBJECTIVE AND IMPORTANCE: We report a unique case of extended leptomeningeal hemangioblastomatosis in a patient presenting with clinical von hippel-lindau disease. CLINICAL PRESENTATION: A 50-year-old male patient had a history of three surgical procedures for the removal of a cerebellar hemangioblastoma, initially considered to be a recurrence of a sporadic form at the same location. Seven years after the last operation, he developed chronic hydrocephalus. Despite a ventriculoperitoneal shunt procedure, he experienced progressive worsening of gait disturbances, associated with touch numbness of the lower limbs and Parinaud's syndrome. magnetic resonance imaging of the brain and spine showed evidence of leptomeningeal contrast enhancement around the brainstem, spinal cord, and cauda equina and enlarged tortuous vessels around the mesencephalon. INTERVENTION: A lumbar laminectomy allowed a leptomeningeal biopsy. Pathological examination revealed leptomeningeal spread of the hemangioblastoma. It is assumed that the tumor arose in the pia mater and that its direction of growth was purely extramedullary, invading all subarachnoid spaces. The patient had a poor outcome as a result of progressive tetraplegia and died 6 months after diagnosis as a result of respiratory failure. CONCLUSION: To the best of our knowledge, the clinical course of our patient, consistent with a thick leptomeningeal spread of hemangioblastoma from the posterior fossa to the sacrum, is unique. Nevertheless, the short life expectancy of our patient is usual in von hippel-lindau disease. This case report illustrates the crucial challenge to develop a specific drug therapy related to angiogenesis in von hippel-lindau disease.
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9/17. Isolated paramedullary hemangioblastoma originating from the first cervical nerve root: case report.

    STUDY DESIGN: A retrospective case of an isolated paramedullary hemangioblastoma originating from the first cervical root is reported. OBJECTIVE: To describe an uncommon type of spinal hemangioblastoma and its operative treatment. SUMMARY OF BACKGROUND DATA: Spinal hemangioblastoma, rare finding accounting for approximately 1.5% to 2.5% of all spinal cord tumors, may have an intramedullary, extramedullary, or extradural location. Cervical hemangioblastomas occur in approximately 45% of the cases and are intramedullary in about 83% of the cases. methods: A 59-year-old man presented with acute subarachnoid hemorrhage in the basal cisterns. Four-vessel angiography showed a highly vascular small tumor at the dorsolateral side of the cervicomedullary junction fed by a branch of the vertebral artery. The lesion was surgically removed. RESULTS: Total removal of the lesion was achieved after identification of both the arterial feeder and the draining vein with the aid of microvascular Doppler sonography. There were no complications, and the patient did well after surgery. CONCLUSIONS: Although hemangioblastomas occurring in the cervicomedullary area usually may cause progressive neural compression, occasionally they also can present clinically as acute subarachnoid hemorrhage. This situation requires urgent and adequate treatment as in the reported case.
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10/17. Proximal nerve root spinal hemangioblastomas: presentation of three cases, MR appearance, and literature review.

    BACKGROUND: Hemangioblastomas (HBLs) are relatively uncommon tumors of uncertain histogenesis usually located in the cerebellum or spinal cord. Much less frequently they are identified in extramedullary locations including the filum terminale, proximal nerve roots, or even distal nerves of the peripheral nervous system (PNS). PNS cases not only present diagnostic challenges but also raise interesting questions regarding the common cell of origin for these CNS and PNS neoplasms. Few studies have detailed the neuroimaging characteristics of the rare extramedullary variants. methods: neuroimaging and intraoperative findings of three recent cases of proximal nerve root HBLs are described. The English language literature on extramedullary HBLs is reviewed and discussed, particularly in regards to magnetic resonance (MR) findings and association with von Hippel-Lindau syndrome (VHL). RESULTS: All 3 of our cases had prominent vessels present within the subarachnoid space on MR scans and all lesions enhanced. All were of intermediate or mildly decreased signal intensity on the T1-weighted images before contrast and were either iso- or hyperintense to spinal cord on the T2-weighted images. Two had probable cystic areas on MR, and all had cystic areas on histologic evaluation. CONCLUSION: These neuroimaging characteristics can serve to distinguish HBLs from the more common benign nerve sheath tumors with which they are most frequently confused. Less than half of all extramedullary HBLs are diagnosed in patients with known VHL. It is important to consider HBLs in the differential diagnosis, as they are vascular and have the propensity for causing significant blood loss at surgery.
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