• Journal of Korean Neurosurgical Society
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• v.29 no.11
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• pp.1523-1526
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• 2000

It has been known that hemangioblastoma of spinal cord occurs about 4% of all the spinal tumors. The authors present a rare case of intradural extramedullary(IDEM) hemangioblastoma of distal spinal cord in 41-year-old male patient. This IDEM mass at the level of conus medullaris showed iso-signal intensities on T1-weighted image(T1-WI) and high-signal intensities on T2-WI, and was enhanced homogeneously on MRI. At surgery, T12- L1 total laminectomy and enbloc mass removal were performed. This IDEM hemangioblastoma was confirmed by histopathologic findings. For less postoperative complication in IDEM tumors, we suggest that precise preoperative evaluation and complete excision via proper surgical approach to distal conus should be reemphasized.

• Journal of Korean Neurosurgical Society
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• v.29 no.6
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• pp.805-809
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• 2000

Hemangioblastoma of the central nervous system rarely occurs in cervicomedullary junction. The unique pathologic features of the tumor involving midline structures are grossly solid in consistency and accompanying extensive spinal cord enlargement. A 63-year-old women presented with progressive right motor weakness and tingling sensation. The MR image showed a well enhancing mass having a cyst and diffuse cord enlargement in the cervicomedullary junction. A total surgical resection was performed and hemangioblastoma was histologicaly verified. Postoperative MR image showed the disappearance of cord enlargement. The right motor weakness was also improved. The authors report a rare case of hemangioblastoma in cervicomedullary junction and the pathophysiology of the spinal cord enlargement are discussed.

• Journal of Korean Neurosurgical Society
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• v.30 no.sup2
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• pp.361-363
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• 2001

Intradural extramedullary hemangioblastoma of the spinal cord is uncommon tumor. Symptom onset is typically in the forth decade. Complete excision offers the best chance for cure. We report a case of extramedullary hemangioblastoma of the spinal cord in old age. A 76 -year old man presented with 1 month history of paraparesis. Multiple enhancing lower thoracic and lumbar spinal masses were seen on magnetic resonance imaging(MRI). Surgical exploration for symptomatic lesion revealed intradural-extramedullary mass, which had hypervascularity. The excised spinal masses were diagnosed as hemangioblastoma. Postoperative course was uneventful and symptoms improved gradually.

• Journal of Korean Neurosurgical Society
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• v.42 no.6
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• pp.436-440
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• 2007

Objective : Spinal cord hemangioblastoma is an uncommon vascular neoplasm with a benign nature and is associated with von Hippel-Lindau (VHL) disease in 20-30% of patients. Total removal of these tumors without significant neurological deficit remains a great challenge. The purpose of this study was to investigate the efficacy of VHL mutation analysis and to evaluate surgical outcome of patients with spinal cord hemangioblastomas. Methods : This study included nine patients treated for spinal cord hemangioblastomas at our institute between December 1994 and March 2006. There were four male and five female patients. Mean age was 37.8 years. The mean follow-up period was 22.4 months. Magnetic resonance imaging (MRI) of the complete neuraxis was done in all cases and VHL mutation analysis was performed in three cases for a definite diagnosis. Results : Six patients had intramedullary tumor, and the remaining patients had intradural extramedullary lesions. Five patients were associated with VHL disease. The von Hippel-Lindau mutation analysis was done in three patients and two of them showed VHL gene abnormality. Tumors were located in the cervical cord in five cases and in the thoracic cord in four cases. All patients underwent surgical intervention, and total removal was achieved in six cases. All patients showed improvement or, at least, clinically stationary state. Surgical complications did not develop in any cases. Conclusion : Spinal hemangioblastoma in this series has been safely and effectively removed via a posterior approach. Postoperatively, clinical outcome was excellent in the majority of cases. The VHL mutation analysis was useful in patients with family history and in those with multiple hemangioblastomas.

• Journal of Korean Neurosurgical Society
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• v.29 no.6
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• pp.836-840
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• 2000

Intramedullary spinal hemagioblastomas usually develope in cervical or thoracic region of spinal cord, but rarely in conus medullaris. We report a case of hemangioblastoma developed in conus medullaris. The 19-year-old male patient presented with slowly progressing low back pain and paresthesia of both legs. MRI and spinal angiography revealed a well-vascularized mass lesion in the conus medullaris with syrinx formation. Total excision of hemangioblastoma was achieved via posterior approach. Postoperatively, patient's walking difficulty was worsened transiently, but it was improved at discharge.

• Journal of Korean Neurosurgical Society
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• v.30 no.8
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• pp.1023-1027
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• 2001

Hemangioblastomas are rare benign tumor of the central nervous system that commonly occur in the posterior fossa around the 4th ventricle. In case of von Hippel-Lindau disease, hemangioblastomas involve multiple regions such as cerebellum, spinal cord and brainstem but, rarely show simultaneous involvement of cerebellum and spinal cord. We have experienced a case of multiple hemangioblastomas that were located at the cerebellum, cervical cord and conus medullaris and also had multiple lesions that a part of von Hippel-Lindau disease ; retinal angioma, syringomyelia, multiple cyst on kidney and pancreas, renal cell carcinoma on left kidney. Hemangioblastomas on cerebellum and spinal cord were removed totally, retinal angioma was treated with laser photocoagulation and renal cell carcinoma was also totally excised. The authors report a case of von Hippel-Lindau disease had multiple located hemangioblastomas on cerebellum, cervical cord and conus medullaris with review of literature.

• Journal of Korean Neurosurgical Society
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• v.30 no.11
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• pp.1291-1299
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• 2001

Objectives : The authors present a retrospective analysis of 14 patients treated for spinal cord hemangioblastoma (SCH) between Dec. 1986 and Mar. 2000. This study was conducted to evaluate and compare the difference of the functional outcomes associated with the extent of surgical removal of SCH. Methods : Eleven patients were male and three patients were female. Their mean age was 37.2 years that ranged from 19 to 62 years. Preoperative magnetic resonance(MR) imaging of the spine was performed in all cases, and preoperative angiography in eleven cases. They were followed from 15 months to 161 months(median follow-up period, 47 months), and we investigated the change of neurological symptoms and functional outcomes with radiological features, especially on MR imaging. Results : Six patients were accompanied by von Hippel-Lindau disease, and three of them had multiple CNS tumors. Thirteen patients had intramedullary tumor, and the remaining one had extradural. Syringomyelia around the tumor was observed in ten cases. All patients underwent surgical removal, and gross total removal(GTR) was achieved in ten cases. Preoperative embolization was performed in four cases. In four patients who were treated with preoperative embolization, intraoperative loss of blood was minimal and GTR was possible. One patient developed a transient swallowing difficulty postoperatively without permanent postoperative neurological deficits. In three of four patients in whom GTR was not possible, their functional outcomes were worsened postoperatively. The functional status at discharge was improved in seven patients, stationary in four patients, and worse in three. At the last follow-up(15-161 months), one of four patients who had been stationary at discharge showed improvement but, the rest did not show any change. All patients who showed neurological improvement were patients with GTR, and the patients with GTR had significant better outcome than those without GTR(p=0.015). Conclusion : Surgical treatment, and especially, GTR is considered as treatment of choice for spinal cord hemangioblastoma. Preoperative embolization may prevent intraoperative bleeding and improve surgical outcome.

• Journal of Korean Neurosurgical Society
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• v.54 no.5
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• pp.415-419
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• 2013

Hemangioblastomas are sporadic tumors found in the cerebellum or spinal cord. Supratentorial hemangioblastomas are rare, and those with meningeal involvement are extremely rare and have been reported in only approximately 130 patients. Here, we report the case of a 51-year-old female patient with supratentorial meningeal hemangioblastoma detected 5 years after surgical resection of an infratentorial hemangioblastoma associated with von Hippel-Lindau disease. Patients with von Hippel-Lindau syndrome are at risk for developing multiple hemangioblastomas, with new tumor formation and growth and possible meningeal infiltration. Regular lifelong follow-up in at-risk patients is recommended and should include the differential diagnosis of dural-based tumors such as angioblastic meningioma and metastatic renal cell carcinoma.

• Journal of Korean Neurosurgical Society
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• v.44 no.2
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• pp.72-77
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• 2008

Objective : The objective of this study was to evaluate the characteristics and surgical outcome of the conus medullaris tumors. Methods : We retrospectively reviewed 26 patients who underwent surgery for conus medullaris tumor from August 1986 to July 2007. We analyzed clinical manifestation, preoperative MRI findings, extent of surgical resection, histopathologic type, adjuvant therapy, and outcomes. Results : There were ependymoma (13), hemangioblastoma (3), lipoma (3), astrocytoma (3), primitive neuroectodermal tumor (PNET) (2), mature teratoma (1), and capillary hemangioma (1) on histopathologic type. Leg pain was the most common symptom and was seen in 80.8% of patients. Pain or sensory change in the saddle area was seen in 50% of patients and 2 patients had severe pain in the perineum and genitalia. Gross total or complete tumor resection was obtained in 80.8% of patients. On surgical outcome. modified JOA score worsened in 26.9% of patients, improved in 34.6%, and remained stable in 38.5%. The mean VAS score was improved from 5.4 to 1.8 among 21 patients who had lower back pain and leg pain. Conclusion : The surgical outcome of conus medullaris tumor mainly depends on preoperative neurological condition and pathological type. The surgical treatment of conus medullaris tumor needs understanding the anatomical and functional characteristics of conus meudllaris tumor for better outcome.

• Journal of Korean Neurosurgical Society
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• v.52 no.3
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• pp.221-227
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• 2012

Objective : Spinal cord hemangioblastomas are rare tumors. Despite their benign, slow-growing nature, they can cause severe neurological consequences. The purpose of this study was to evaluate variable factors, including clinical features, tumor findings, the extent of resection, and its recurrence or progression, which determine postoperative functional outcomes. Methods : This study included sixteen patients at our institute who underwent microsurgical resection for sporadic spinal intramedullary hemangioblastomas and spinal intramedullary hemangioblastomas associated with von Hippel-Lindau (VHL) disease, between June 2003 and March 2012. Results : A total of 30 operations were performed. Total resection (TR) of the tumor was achieved in 10 patients, and subtotal resection (STR) was achieved in 6. Postoperatively, the initial presenting symptoms were improved in 18.7% of the patients and were unchanged in 56.3%, but 25% were worse. Stable postoperative neurological functions were found in 83% of patients with preoperative McCormick grade I, and TR was achieved in 75% of these patients. In the STR group, poorer neurological status was observed in one patient, despite multiple operations. There were no poorer outcomes in the four cases of VHL disease. Various factors were analyzed, but only a correlation between the pre- and postoperative neurological status was verified in the TR-group patients. Conclusion : Preoperative focal neurological impairment and meticulous microsurgical manipulation may be predictors of favorable outcomes for solitary hemangioblastomas. In addition, the preservation of function is more important than the extent of resection in VHL disease.