• 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.

• 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.40 no.3
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• pp.164-168
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• 2006

Objective : The goal of treatment for spinal cord ependymoma is complete removal without postoperative neurological deficit. The authors analyzed the surgical results and factors influencing the postoperative prognosis. Methods : Fifty-one cases of primary spinal cord ependymoma, surgically treated between 1979 and 2003, were retrospectively analyzed. The mean follow-up period was 44 months. Results : Gross total removal was achieved in 42 patients and incomplete removal in nine. The proportion of complete surgical removals was influenced by tumor location and histology. Disease progression was observed in five cases [9.8%], the mean progression free interval after surgical removal was 48 months and the 5-year progression free rate was 68%. Disease progression was found in none of the 42 cases who underwent complete removal, and in 5 of 9 cases who hadincomplete removal group [P<0.001]. Statistically significant disease-progression factors by multivariate analysis were the surgical extent of removal [P=0.012]. preoperative functional status [P=0.032] the presence of intratumoral cysts [P=0.007] and postoperative radiation therapy [P=0042]. Of those patients who underwent incomplete removal, radiation therapy was found to significantly improve the clinical result [P=0042]. Conclusion : In the surgical treatment of spinal cord ependymoma, preoperative functional status, the presence of intratumoral cysts, the extent of removal, and postoperative radiation therapy were found to be significant prognostic factors of postoperative outcome.

• Journal of Korean Neurosurgical Society
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• v.53 no.5
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• pp.316-319
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• 2013

Neuromyelitis optica (NMO) is considered to be a rarer autoimmune disease than multiple sclerosis. It is very difficult to make a diagnosis of MNO for doctors who are not familiar with its clinical features and diagnostic criteria. We report a case of a young female patient who had been suffering motor weakness and radiating pain in both upper extremities. Cervical MRI showed tumorous lesion in spinal cord and performed surgery to remove lesion. We could not find a tumor mass in operation field and final diagnosis was NMO. NMO must be included in the differential diagnosis of lesions to rescue the patient from invasive surgical interventions. More specific diagnostic tools may be necessary for early diagnosis and proper treatment.

• 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.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 Physiology & Pathology in Korean Medicine
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• v.21 no.6
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• pp.1663-1669
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• 2007

Acute transverse myelitis(ATM) is defined as an acute intramedullary dysfunction of the spinal cord, ascendng or static involving both halves of the cord and appearing without any history of previous neurological diseases due to traumatic accident, tumor of all kind, encephalitis and of course excluding all possible viral, bacterial and fungal infection. It is mainly characterized by acute motor disorder of both limbs in respect to which spinal segments are affected as well as sensory disorder and dysuria & dyschezia. The exact cause is unknown, however it is recently suggested that immunological factors are highly involved. It has been reported by several reliable sources that it is often accompanied by immunological diseases such as systemic lupus erythematosus(SLE). As treatments non steroid anti-inflammatory drugs(NSAIDS) are primarily recommended as to steroids, limited doses are injected only with the proper prescription from the physician. Operative methods are not options as traumatic accidents and tumors are excluded as factors. To enhance muscle strength and prevent articular contracture physical therapy and passive exercise is imperative. The following patient whose chief complaints were mainly about hypoesthesia of Rt. lower limb and stiffness of phalanges of both fingers as well as to weakness of lower extermity. Therefore it has been diagnosed as arthalgia syndrome. In oriental medicine factors such as wind evil heat-evil, dampness-heat evil, cold evil cause the arthalgia syndrome. In this case the patient was diagnosed as dampness-heat evil and herbal medicine Chunglijagam-Tang and Dong-Qi acupuncture was applied to treat bladder disorder.

• Journal of the Korean Society of Radiology
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• v.79 no.5
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• pp.294-301
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• 2018

Subependymomas are rare benign central nervous system tumor which account of 0.7% of all intracranial tumors. Subependymomas show characteristic MR findings according to their location. However, sometimes atypical findings could be found. In addition, subependymomas can occur very rarely in the spinal cord. We report two cases of pathologically confirmed subependymoma, one of which shows atypical appearances in spite of their intraventicular location and the other of which shows rare case of spinal intramedullary subependymoma. We review the clinical symptoms and radiologic findings of two cases.

• Journal of Korean Neurosurgical Society
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• v.64 no.4
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• pp.592-607
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• 2021

Objective : Few studies exist on primary spinal cord tumors (PSCTs) in pediatric patients. The purpose of this study was to perform descriptive analysis and detailed survival analysis for PSCTs. Methods : Between 1985 and 2017, 126 pediatric patients (male : female, 56 : 70) with PSCTs underwent surgery in a single institution. We retrospectively analyzed data regarding demographics, tumor characteristics, outcomes, and survival statistics. Subgroup analysis was performed for the intramedullary (IM) tumors and extradural (ED) tumors separately. Results : The mean age of the participants was 6.4±5.04 years, and the mean follow-up time was 69.5±46.30 months. The most common compartment was the ED compartment (n=57, 45.2%), followed by the IM (n=43, 34.1%) and intradural extramedullary (IDEM; n=16, 12.7%) compartments. Approximately half of PSCTs were malignant (n=69, 54.8%). The most common pathologies were schwannomas (n=14) and neuroblastomas (n=14). Twenty-two patients (17.5%) died from the disease, with a mean disease duration of 15.8±15.85 months. Thirty-six patients (28.6%) suffered from progression, with a mean period of 22.6±30.81 months. The 10-year overall survival (OS) rates and progression-free survival (PFS) rates were 81% and 66%, respectively. Regarding IM tumors, the 10-year OS rates and PFS rates were 79% and 57%, respectively. In ED tumors, the 10-year OS rates and PFS rates were 80% and 81%, respectively. Pathology and the extent of resection showed beneficial effects on OS for total PSCTs, IM tumors, and ED tumors. PFS was affected by both the extent of removal and pathology in total PSCTs and ED tumors; however, pathology was a main determinant of PFS rather than the extent of removal in IM tumors. The degree of improvement in the modified McCormick scale showed a trend towards improvement in patients with IM tumors who achieved gross total removal (p=0.447). Conclusion : Approximately half of PSCTs were malignant, and ED tumors were most common. The most common pathologies were schwannomas and neuroblastomas. Both the pathology and extent of resection had a decisive effect on OS. For IM tumors, pathology was a main determinant of PFS rather than the extent of removal. Radical excision of IM tumors could be a viable option for better survival without an increased risk of worse functional outcomes.