• Journal of Korean Neurosurgical Society
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• 제52권4호
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• pp.417-419
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• 2012

Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clinical presentation, pathogenesis, and treatment. A male infant was delivered normally, with uneventful development. At 16 days post-delivery, his family took him to a pediatrician because of a mass on his upper back. Magnetic resonance imaging of the thoracic spine revealed a well-demarcated soft-tissue mass with central cystic change or necrosis at the subcutaneous layer of the posterior back (T2-7 level). Another mass was found with a fat component at the spinal canal of the T1-3 level, which was intradural extramedullary space. After six weeks, the spinal cord tumor and subcutaneous mass were grossly total resected; pathologic findings indicated an atypical myxoid spindle cell neoplasm, possibly nerve sheath in type. The final diagnosis of the mass was an atypical myxoid spindle cell neoplasm. The postoperative course was uneventful, and the patient was discharged after nine days without any neurological deficit. We report a rare case of an intradural extramedullary spinal tumor with subcutaneous mass in a neonate. It is necessary to monitor the patient's status by examining consecutive radiologic images, and the symptoms and neurological changes should be observed strictly during long-term follow-up.

• Journal of Korean Neurosurgical Society
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• 제39권5호
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• pp.385-388
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• 2006

An Intramedullary subependymoma of the spinal cord is a rare tumor with only 43 reported cases in the literature. Most of them are reported to be localized within the cervical spinal cord. We report a rare case of a thoracic spine intramedullary subependymoma in a 37-year-old female who presented with back pain and radiating leg pain. Subtotal resection and post-operative radiotherapy were performed. Subependymomas developing in the spinal cord are benign with a low proliferative potential. Complete resection of the tumor appears to be the optimal method for a complete cure. However aggressive surgery may cause severe neurological deficit. Therefore, if severe neurological deficits are expected after complete removal, a partial removal and postoperative radiation therapy is an alternative method for treatment in selected cases. A large-scale randomized study is mandatory to clarify the effectiveness of radiotherapy and to establish the recurrence rate and prognosis with respect to the surgical removal of these tumors.

• Journal of Korean Neurosurgical Society
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• 제44권2호
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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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• 제61권2호
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• pp.233-242
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• 2018

Objective : A spinal cord subependymoma is an uncommon, indolent, benign spinal cord tumor. It is radiologically similar to a spinal cord ependymoma, but surgical findings and outcomes differ. Gross total resection of the tumor is not always feasible. The present study was done to determine the clinical, radiological and pathological characteristics of spinal cord subependymomas. Methods : We retrospectively reviewed the medical records of ten spinal cord subependymoma patients (M : F=4 : 6; median 38 years; range, 21-77) from four institutions. Results : The most common symptoms were sensory changes and/or pain in eight patients, followed by motor weakness in six. The median duration of symptoms was 9.5 months. Preoperative radiological diagnosis was ependymoma in seven and astrocytoma in three. The tumors were located eccentrically in six and were not enhanced in six. Gross total resection of the tumor was achieved in five patients, whereas subtotal or partial resection was inevitable in the other five patients due to a poor dissection plane. Adjuvant radiotherapy was performed in two patients. Neurological deterioration occurred in two patients; transient weakness in one after subtotal resection and permanent weakness after gross total resection in the other. Recurrence or regrowth of the tumor was not observed during the median 31.5 months follow-up period (range, 8-89). Conclusion : Spinal cord subependymoma should be considered when the tumor is located eccentrically and is not dissected easily from the spinal cord. Considering the rather indolent nature of spinal cord subependymomas, subtotal removal without the risk of neurological deficit is another option.

• Journal of Korean Neurosurgical Society
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• 제42권6호
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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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• 제64권4호
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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.

• Journal of Korean Neurosurgical Society
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• 제64권4호
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• pp.671-673
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• 2021

• Journal of Korean Neurosurgical Society
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• 제49권5호
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• pp.302-304
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• 2011

Thoracic intramedullary schwannomas are rare spinal cord tumors. Most of these tumors have been reported as a single lesion in the spinal cord. The authors report the first case of intramedullary schwannoma accompanying by extramedullary beads-like daughter masses of the thoracic spine. A 68-year-old male presented with walking disturbance and decreased sensation below T10 dermatome. Imaging workup revealed an intramedullary mass at T6 and T7, T6 and T7 laminectomy and mass removal were performed. Intraoperatively, extramedullary beads-like daughter masses along the nerve roots adjacent to intramedullary mass were identified. Total removal of intramedullary lesion and partial resection of extramedullary masses were done. Histological analysis confirmed the diagnosis of schwannoma. The patient could ambulate independently at postoperative 1 month without any neurological sequelae. The authors experienced a surgical case of intramedullary schwannoma accompanying by extramedullary beads-like same pathologies in the thoracic spine.

• Annals of Clinical Neurophysiology
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• 제22권2호
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• pp.109-111
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• 2020

Germ-cell tumors (GCTs) are common in the central nervous system. GCTs are highly sensitive to radiotherapy and chemotherapy and can be cured without radical surgery. However, this tumor produces nonspecific imaging findings, and its rarity can make diagnosis challenging. Here we report a case in which the first diagnosis was uncertain and follow-up imaging revealed intramedullary germinoma. The patient underwent chemotherapy and radiotherapy after the diagnosis.

• Journal of Korean Neurosurgical Society
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• 제39권3호
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• pp.224-227
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• 2006

Extradural lipomas have been frequently reported in the literature, but intramedullary lipomas are far rarer, constituting only approximately 2% of total intramedullary tumors. Intramedullary lipomas are also commonly associated with spinal dysraphism. Lipomas which are not associated with spinal dysraphism are present in only about 1% of spinal lipoma patients. Here, we report a rare case of a patient suffering from an isolated intramedullary lipoma without evidence of spinal dysraphism.