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1.  Clinical and Neuroimaging Outcomes of Surgically Treated Intracranial Cysts in 110 Children 
Objective
The indications and optimal surgical treatments for intracranial cysts are controversial. In the present study, we describe long-term clinical and neuroimaging results of surgically treated intracranial cysts in children. The goal of this study is to contribute to the discussion of the debate.
Methods
This study included 110 pediatric patients that underwent surgeries to treat intracranial cysts. Endoscopic cyst fenestrations were performed in 71 cases, while craniotomies and cyst excisions (with or without fenestrations) were performed in 30 patients. Cystoperitoneal shunts were necessary for nine patients. Long-term results were retrospectively assessed with medical and neuroimaging records.
Results
Clinical and radiological improvement was reported in 87.3% and 92.8% of cases, respectively, after endoscopic neurosurgery, and in 93.3% and 100% using open microsurgery whereas 88.9% and 85.7% after shunt operation. There were no statistical differences in clinical outcomes (p=0.710) or volume reductions (p=0.177) among the different surgeries. There were no mortalities or permanent morbidities, but complications such as shunt malfunctions, infections, and subdural hematomas were observed in 56% of the patients that had shunt operations. A total of 13 patients (11.8%) underwent additional surgeries due to recurrences or treatment failures. The type of surgery performed did not influence the recurrence rate (p=0.662) or the failure rate (p=0.247).
Conclusion
Endoscopic neurosurgeries are less invasive than microsurgeries and are at least as effective as open surgeries. Thus, given the advantages and complications of these surgical techniques, we suggest that endoscopic fenestration should be the first treatment attempted in children with intracranial cysts.
doi:10.3340/jkns.2012.52.4.325
PMCID: PMC3488640  PMID: 23133720
Arachnoid cysts; Central nervous system cysts; Congenital; Neuroendoscopy; Child
2.  Prognostic Factors and Therapeutic Outcomes in 22 Patients with Pleomorphic Xanthoastrocytoma 
Objective
Pleomorphic xanthoastrocytoma (PXA) is a rare primary low-grade astrocytic tumor classified as WHO II. It is generally benign, but disease progression and malignant transformation have been reported. Prognostic factors for PXA and optimal therapies are not well known.
Methods
The study period was January 2000 to March 2012. Data on MR findings, histology, surgical extents and adjuvant therapies were reviewed in twenty-two patients diagnosed with PXA.
Results
The frequent symptoms of PXA included seizures, headaches and neurologic deficits. Tumors were most common in the temporal lobe followed by frontal, parietal and occipital lobes. One patient who died from immediate post-operative complications was excluded from the statistical analysis. Of the remaining 21 patients, 3 (14%) died and 7 (33%) showed disease progression. Atypical tumor location (p<0.001), peritumoral edema (p=0.022) and large tumor size (p=0.048) were correlated with disease progression, however, Ki-67 index and necrosis were not statistically significant. Disease progression occurred in three (21%) of 14 patients who underwent GTR, compared with 4 (57%) of 7 patients who did not undergo GTR, however, it was not statistically significant. Ten patients received adjuvant radiotherapy and the tumors were controlled in 5 of these patients.
Conclusion
The prognosis for PXA is good; in our patients overall survival was 84%, and event-free survival was 59% at 3 years. Atypical tumor location, peritumoral edema and large tumor size are significantly correlated with disease progression. GTR may provide prolonged disease control, and adjuvant radiotherapy may be beneficial, but further study is needed.
doi:10.3340/jkns.2013.53.5.281
PMCID: PMC3730029  PMID: 23908701
Pleomorphic xanthoastrocytoma; Prognosis; outcome; Radiotherapy
3.  Integrative genomic analyses identify LIN28 and OLIG2 as markers of survival and metastatic potential in childhood central nervous system primitive neuro-ectodermal brain tumours 
The lancet oncology  2012;13(8):838-848.
Background
Childhood Central Nervous System Primitive Neuro-Ectodermal brain Tumours (CNS-PNETs) are highly aggressive brain tumours for which molecular features and best therapeutic strategy remains unknown. We interrogated a large cohort of these rare tumours in order to identify molecular markers that will enhance clinical management of CNS-PNET.
Methods
Transcriptional and copy number profiles from primary hemispheric CNS-PNETs were examined using clustering, gene and pathways enrichment analyses to discover tumour sub-groups and group-specific molecular markers. Immuno-histochemical and/or gene expression analyses were used to validate and examine the clinical significance of novel sub-group markers in 123 primary CNS-PNETs.
Findings
Three molecular sub-groups of CNS-PNETs distinguished by primitive neural (Group 1), oligo-neural (Group 2) and mesenchymal lineage (Group 3) gene expression signature were identified. Tumour sub-groups exhibited differential expression of cell lineage markers, LIN28 and OLIG2, and correlated with distinct demographics, survival and metastatic incidence. Group 1 tumours affected primarily younger females; male: female ratios were respectively 0.61 (median age 2.9 years; 95% CI: 2.4–5.2; p≤ 0.005), 1.25 (median age 7.9 years; 95% CI: 6–9.7) and 1.63 (median age 5.9 years; 95% CI: 4.9–7.8) for group 1, 2 and 3 patients. Overall outcome was poorest in group 1 patients which had a median survival of 0.8 years (95% CI: 0.47–1.2; p=0.019) as compared to 1.8 years (95% CI: 1.4–2.3) and 4.3 years; (95% CI: 0.82–7.8) respectively for group 2 and 3 patients. Group 3 tumours had the highest incidence of metastases at diagnosis; M0: M+ ratio were respectively 0.9 and 3.9 for group 3, versus group 1 and 2 tumours combined (p=0.037).
Interpretation
LIN28 and OLIG2 represent highly promising, novel diagnostic and prognostic molecular markers for CNS PNET that warrants further evaluation in prospective clinical trials.
doi:10.1016/S1470-2045(12)70257-7
PMCID: PMC3615440  PMID: 22691720
4.  Validation of Pediatric Functional Assessment of Cancer Therapy Questionnaire (Version 2.0) in Brain Tumor Survivor Aged 13 Years and Older (Parent Form)(PedsFACT-BrS Parent of Adolescent) 
Objective
The aim of this study was to evaluate the reliability and validity of the Pediatric Functional Assessment of Cancer Therapy Questionnaire Brain Tumor Survivor (version 2.0) Aged 13 years and older (Parent Form) (pedsFACT-BrS parent of adolescent).
Methods
The pedsFACT-BrS parent of adolescent was translated and cross-culturally adapted into Korean, following standard Functional Assessment of Chronic Illness Therapy (FACIT) methodology. The psychometric properties of the pedsFACT-BrS parent of adolescent were evaluated in 170 brain tumor patient's mothers (mean age=43.38 years). Pretesting was performed in 30 mothers, and the results indicated good symptom coverage and overall comprehensibility. The participants also completed the Child Health Questionnaire Parent Form 50 (CHQ-PF-50), Neuroticism in Eysenck Personality Questionnaire, and Karnofsky score.
Results
In validating the pedsFACT-BrS parent of adolescent, we found high internal consistency, with Cronbach's α coefficients ranging from 0.76 to 0.94. The assessment of test-retest reliability using intraclass correlation coefficient revealed satisfactory values with ICCs ranging from 0.84 to 0.93. The pedsFACT-BrS for parent of adolescent also demonstrated good convergent and divergent validities when correlated with the Child Health Questionnaire Parent Form 50 (CHQ-PF-50) and the Neuroticism in Eysenck Personality Questionnaire. The pedsFACT-BrS parent of adolescent showed good clinical validity, and effectively differentiated between clinically distinct patient groups according to the type of treatment, tumor location, shunt, and Karnofsky score of parent proxy report.
Conclusion
We confirmed that this reliable and valid instrument can be used to properly evaluate the quality of life of Korean adolescent brain tumor patients by their parents' proxy report.
doi:10.3340/jkns.2011.49.3.147
PMCID: PMC3085809  PMID: 21556233
Quality of life; Adolescent brain tumor patient; pedsFACT-BrS parent of adolescent; Validity
5.  The role of the membrane cytoskeleton cross-linker ezrin in medulloblastoma cells 
Neuro-Oncology  2009;11(4):381-393.
Medulloblastoma is a highly malignant brain tumor that occurs predominantly in children. The molecular pathogenesis of medulloblastoma is under investigation. Previously, we used complementary DNA micro-array analysis to compare patterns of gene expression in medulloblastoma samples versus normal cerebellum. The cytoskeletal protein ezrin was found to be overexpressed in medulloblastoma compared with normal cerebellum, an observation that was further validated by immunohistochemistry and real-time PCR analysis. To assess the role of ezrin in medulloblastoma, we studied ezrin’s role in medulloblastoma migration, invasion, and adhesion. Western blotting and immunofluorescence showed high expression of ezrin in four medulloblastoma cell lines, and ezrin was primarily localized to filopodia. Ezrinspecific small interfering RNA suppressed the formation of filopodia and in vitro migration, invasion, and adhesion. We also used a stably transfected medulloblastoma cell line to study the effect of ezrin overexpression. We showed that high expression of ezrin promotes filopodia formation and in vitro invasion. Finally, athymic mice implanted with ezrin-overexpressing DAOY medulloblastoma cell clones in the cerebellum showed shortened survival compared with controls. These findings suggest that, in addition to other cytoskeletal proteins, ezrin plays an important role in medulloblastoma adhesion, migration, and invasion.
doi:10.1215/15228517-2008-110
PMCID: PMC2743218  PMID: 19088174
cytoskeleton; ezrin; invasion; medulloblastoma; siRNA
6.  Genetic and Epigenetic Inactivation of Kruppel-like Factor 4 in Medulloblastoma1 
Neoplasia (New York, N.Y.)  2010;12(1):20-27.
Although medulloblastoma is the most common pediatric malignant brain tumor, its molecular underpinnings are largely unknown. We have identified rare, recurrent homozygous deletions of Kruppel-like Factor 4 (KLF4) in medulloblastoma using high-resolution single nucleotide polymorphism arrays, digital karyotyping, and genomic real-time polymerase chain reaction (PCR). Furthermore, we show that there is loss of physiological KLF4 expression in more than 40% of primary medulloblastomas both at the RNA and protein levels. Medulloblastoma cell lines drastically increase the expression of KLF4 in response to the demethylating agent 5-azacytidine and demonstrate dense methylation of the promoter CpG island by bisulfite sequencing. Methylation-specific PCR targeting the KLF4 promoter demonstrates CpG methylation in approximately 16% of primary medulloblastomas. Reexpression of KLF4 in the D283 medulloblastoma cell line results in significant growth suppression both in vitro and in vivo. We conclude that KLF4 is inactivated by either genetic or epigenetic mechanisms in a large subset of medulloblastomas and that it likely functions as a tumor suppressor gene in the pathogenesis of medulloblastoma.
PMCID: PMC2805880  PMID: 20072650
7.  Comparative Analysis of Cervical Arthroplasty Using Mobi-C® and Anterior Cervical Discectomy and Fusion Using the Solis® -Cage 
Objective
Although anterior cervical discectomy and fusion (ACDF) is the standard treatment for degenerative cervical disc disease, concerns regarding adjacent level degeneration and loss of motion have suggested that arthroplasty may be a better alternative. We have compared clinical and radiological results in patients with cervical disc herniations treated with arthroplasty and ACDF.
Methods
We evaluated 53 patients treated for cervical disc herniations with radiculopathy, 21 of whom underwent arthroplasty and 32 of whom underwent ACDF. Clinical results included the Visual Analogue Scale (VAS) score for upper extremity radiculopathy, neck disability index (NDI), duration of hospital stay and convalescence time. All patients were assessed radiologically by measuring cervical lordosis, segmental lordosis and segmental range-of-movement (ROM) of operated and adjacent disc levels.
Results
Mean hospital stay (5.62 vs. 6.26 days, p<0.05) and interval between surgery and return to work (1.10 vs. 2.92 weeks, p<0.05) were significantly shorter in the arthroplasty than in the fusion group. Mean NDI and extremity VAS score improved after 12 months in both groups. Although it was not significant, segmental ROM of adjacent levels was higher in the fusion group than in the arthroplasty group. And, segmental motion of operated levels in arthroplasty group maintained more than preoperative value at last follow up.
Conclusion
Although clinical results were similar in the two groups, postoperative recovery was significantly shorter in the arthroplasty group. Although it was not significant, ROM of adjacent segments was less in the arthroplasty group. Motion of operated levels in arthroplasty group was preserved at last follow up.
doi:10.3340/jkns.2008.44.4.217
PMCID: PMC2588324  PMID: 19096680
Anterior discectomy and fusion (ACDF); Arthroplasty; Cervical disc herniations; Artificial cervical disc; Mobi-C®; Solis cages®
8.  Postoperative Electroencephalogram for Follow up of Pediatric Moyamoya Disease 
Journal of Korean Medical Science  2006;21(3):495-499.
It is well known that the electroencephalographic finding in patients with moyamoya disease demonstrates the characteristic "re-build up" phenomenon a few minutes after hyperventilation. To evaluate the usefulness of an electroencephalogram (EEG) in the postoperative management of children with moyamoya disease, we studied the presence or absence of improvement in the clinical, single photon emission computed tomography (SPECT) and EEG findings, before and after surgery. Twenty-two patients, who underwent indirect revascularization surgery for moyamoya disease, were included in our study. Clinical improvement was assessed as the disappearance or decrease of a transient ischemic attack or headache. The findings on the EEG and SPECT were considered improved when the re-build up phenomenon was absent and when there was improvement in the cerebrovascular reserve as a result of the acetazolamide challenge test. The statistical correlation analysis for both clinical and EEG improvement were consistent (kappa value=0.409, p<0.05). However, the result from the clinical and SPECT improvement as well as that from EEG and SPECT improvement were not statistically significant. Our results suggest that EEG can be used as a noninvasive and simple follow-up test for moyamoya disease after indirect revascularization surgery if the hyperventilation procedure is effectively performed during EEG recording.
doi:10.3346/jkms.2006.21.3.495
PMCID: PMC2729958  PMID: 16778396
Moyamoya Disease; Electroencephalography; Follow-up Studies
9.  Localized 1H-MR Spectroscopy in Moyamoya Disease before and after Revascularization Surgery 
Korean Journal of Radiology  2003;4(2):71-78.
Objective
To evaluate, using localized proton magnetic resonance spectroscopy (1H-MRS), the cerebral metabolic change apparent after revascularization surgery in patients with moyamoya disease.
Materials and Methods
Sixteen children with moyamoya disease and eight age-matched normal controls underwent MR imaging, MR angiography, conventional angiography, and 99mTc-ECD SPECT. Frontal white matter and the basal ganglia of both hemispheres were subjected to localized 1H-MRS, and after revascularization surgery, four patients underwent follow-up 1H-MRS.
Results
Decreased NAA/Cr ratios (1.35±0.14 in patients vs. 1.55±0.24 in controls) and Cho/Cr ratios (0.96±0.13 in patients vs. 1.10±0.11 in controls) were observed in frontal white matter. After revascularization surgery, NAA/Cr and Cho/Cr ratios in this region increased. In the basal ganglia, there is no abnormal metabolic ratios.
Conclusion
Localized 1H-MRS revealed abnormal metabolic change in both hemispheres of children with moyamoya disease. Because of its non-invasive nature, 1H-MRS is potentially useful for the preoperative evaluation of metabolic abnormalities and their postoperative monitoring.
doi:10.3348/kjr.2003.4.2.71
PMCID: PMC2698073  PMID: 12845301
Magnetic resonance (MR), spectroscopy; Brain, infarction; Moyamoya disease
10.  Reduced-dose craniospinal radiotherapy followed by high-dose chemotherapy and autologous stem cell rescue for children with newly diagnosed high-risk medulloblastoma or supratentorial primitive neuroectodermal tumor 
The Korean Journal of Hematology  2010;45(2):120-126.
Background
In this study, we investigated the effects of reduced-dose craniospinal radiotherapy (CSRT) followed by tandem high-dose chemotherapy (HDCT) with autologous stem cell rescue (ASCR) in children with a newly diagnosed high-risk medulloblastoma (MB) or supratentorial primitive neuroectodermal tumor (sPNET).
Methods
Between March 2005 and April 2007, patients older than 3 years with a newly diagnosed high-risk MB or sPNET were enrolled. The patients received two cycles of pre-RT chemotherapy consisting of cisplatin, etoposide, vincristine, and cyclophosphamide (cycle A), and carboplatin, etoposide, vincristine, and ifosphamide (cycle B), followed by CSRT with 23.4 Gy and local RT with 30.6 Gy. After four cycles of post-RT chemotherapy (cycles A, B, A, and B), tandem double HDCT with ASCR was performed.
Results
A total of 13 patients (MB=11, sPNET=2) were enrolled. Of these, one patient progressed, one patient died of septic shock after the second cycle of B, and one patient relapsed after the third cycle of B. The 3-year event-free survival (EFS) rate of the patients intended for HDCT was 76.9%, whereas the 3-year EFS rate of the patients who received HDCT was 100%. No treatment-related mortality occurred during HDCT.
Conclusion
Although the follow-up period was short and the patient cohort was small in size, the results of this study are encouraging. The limited toxicity and favorable EFS rate observed in children treated with reduced-dose CSRT followed by HDCT and ASCR warrant further exploration in a larger study population.
doi:10.5045/kjh.2010.45.2.120
PMCID: PMC2983022  PMID: 21120191
Radiotherapy; High-dose chemotherapy; Autologous stem cell transplantation; Medulloblastoma; Supratentorial primitive neuroectodermal tumor; Children

Results 1-10 (10)