• Brain Tumor Research and Treatment
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• v.6 no.2
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• pp.60-67
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• 2018

Background Recently, modern technology such as diffusion tensor imaging (DTI), neuro-navigation and intraoperative neurophysiological monitoring (IOM) have been actively adopted for the treatment of thalamic tumors. We evaluated surgical outcomes and efficacy of the aforementioned technologies for the treatment of pediatric thalamic tumors. Methods We retrospectively reviewed clinical data from 37 children with thalamic tumors between 2004 and 2017. There were 44 operations (27 tumor resections, 17 biopsies). DTI was employed in 17 cases, neuro-navigation in 23 cases and IOM in 14 cases. All diagnoses were revised according to the 2016 World Health Organization Classification of Tumors of the Central Nervous System. Progression-free survival (PFS) and overall survival (OS) rates were calculated, and relevant prognostic factors were analyzed. The median follow-up duration was 19 months. Results Fifteen cases were gross total resections (GTR), 6 subtotal resections (STR), and 6 partial resections (PR). Neurological status did not worsen after 22 tumor resections. There were statistically significant differences in terms of the extent of resection between the groups with DTI, neuro-navigation and IOM (n=12, GTR or STR=12) and the group without at least one of the three techniques (n= 15, GTR or STR=9, p=0.020). The mean PFS was $87.2{\pm}38.0$ months, and the mean OS $90.7{\pm}36.1$ months. The 5-year PFS was 37%, and the 5-year OS 47%. The histological grade ($p{\leq}0.001$) and adjuvant therapy (done vs. not done, p=0.016) were significantly related to longer PFS. The histological grade (p=0.002) and the extent of removal (GTR/STR vs. PR/biopsy, p=0.047) were significantly related to longer OS. Conclusion Maximal surgical resection was achieved with acceptable morbidity in children with thalamic tumors by employing DTI, neuro-navigation and IOM. Maximal tumor resection was a relevant clinical factor affecting OS; therefore, it should be considered the initial therapeutic option for pediatric thalamic tumors.

• Child Health Nursing Research
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• v.9 no.1
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• pp.9-17
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• 2003

Families of children with cancer face many illness-related demands. The perceived social support is a critical resource for the family adaptation process. And the patterns of family adaptation to childhood cancer varies as characteristics of disease, which is prognosis, the influence of cognition function, and treatment process. The conceptual definition of social support is not unidimensional. However, most studies focus on general aspect of mediating effects on adaptation. Diverse dimensions of perceived social support should be considered in its effectiveness for intervention. Therefore, this study was undertaken to determine whether family's perceived social support influences the family adaptation of family with pediatric cancer and what dimension influences mostly in family adaptation as the characteristics of disease in the family of children with cancer. The subject was consisted of 102 families with pediatric cancer who had been diagnosed as leukemia or brain tumor last 2 years. Those families had participated in the education program or meetings for family who have with pediatric cancer children. The measurement for this study were Personal Resource Questionnaire (PRQ) Part-Ⅱ developed by Brandt and Weinert to measure parents' perceived social support, and the McCubbin's Coping Health Inventory for Parents (CHIP) to measure family coping. The results of this study can be summarized as follows; Regression analysis showed that perceived social support has effect on family adaptation with β=.43, p<.01. In the group of family of child with leukemia, social support as general has effect on adaptation (β=.40, p<.01) and specially, social support perceived as intimacy was strongly effect on family adaptation. And In the group of brain tumor, Social support has effect on adaptation(β=.46, p<.01) and among the social support domains, the self esteem dimension was most predictable to family adaptation. In conclusion, the perceived social support is a predictor on family adaptation and useful vehicle to help family who has child with pediatric cancer. An important clinical implication is that specified support program for intervention may be useful and critical for the family who has diverse pediatric conditions of childhood cancer. Further studies should stress the effects of family support for clinical intervention and is needed with diverse stage of development and pediatric conditions.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.215-220
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• 2003

A 14-month-old child visited emergency room with stuporous mental state. He had been suffering from failure to thrive (FTT) and emaciation since three months of age, but he had good appetite and had been euphoric. A large mass was found in the third ventricle by brain CT and MRI. His parents refused operative removal of the mass and he expired 14 days after admission. It is necessary to include diencephalic syndrome in the differential diagnosis of failure to thrive when infants show FTT despite of good appetite and euphoric status.

• Clinical and Experimental Pediatrics
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• v.51 no.12
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• pp.1363-1367
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• 2008

Cavernous hemangioma can occur in the entire brain but rarely in cerebellum, especially in the pediatric age group. Headache, seizure, gait disturbance, recurrent bleeding may be seen. This tumor is a relatively benign condition but if the lesion located in the posterior fossa or the brain stem bleeds, irreversible brain damage may occur because of its restrictive space. Moreover, it must be differentiated from malignant tumors. We report 12.6 year-old boy who represented posterior neck myalgia as the presenting symptom. The pain continued for about a month despite analgesic medications. Brain MRI showed intracranial hemorrhage in the left cerebellum (4.5 cm) representing repeated hemorrhages at different times and originated from the cavernous hemangioma accompanied by mild hydrocephalus. The lesion was surgically removed successfully and the cavernous hemangioma was confirmed by pathologic findings. After the follow-up period of 14 months, he is in good condition without any complications.

• Journal of Korean Neurosurgical Society
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• v.61 no.3
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• pp.333-342
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• 2018

Intracranial germ cell tumors (iGCTs) are a heterogeneous group of tumors with peculiar characteristics clearly distinguished from other brain tumors of neuroepithelial origin. Diverse histology, similarity to gonadal GCT, predilection to one sex, and geographic difference in incidence all present enigmas and fascinating challenges. The treatment of iGCT has advanced for germinoma to date; thus, clinical attention has shifted from survival to long-term quality of life. However, for non-germinomatous GCT, current protocols provide only modest improvement and more innovative therapies are needed. Recently, next-generation sequencing studies have revealed the genomic landscape of iGCT. Novel mutations in the KIT-RAS-MAPK and AKT-MTOR pathways were identified. More importantly, methylation profiling revealed a new method to assess the pathogenesis of iGCT. Molecular research will unleash new knowledge on the origin of iGCT and solve the many mysteries that have lingered on this peculiar neoplasm for a long time.

• Advances in pediatric surgery
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• v.13 no.2
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• pp.169-178
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• 2007

Teratomas arising from totipotential primitive germ cell are composed of 2 or 3 germ cell layers. We reviewed the records of 166 children who underwent the operation for teratoma from Jan, 1990 through April, 2007. There were 40 boys and 126 girls (average age $6.93{\pm}5.83$ years). Primary sites were ovary (n = 88), sacrococcygeum (n = 24), testis (n = 16), retroperitoneum (n = 16), mediastinum (n = 8), brain (n = 4), thymus (n = 3) and a single tumor involved the adrenal gland, neck, middle ear, oropharynx, stomach, pelvis, omentum, chest wall and scalp. Teratomas were mature in 141 patients, and immature in 25. Six patients who had ovarian immature teratomas grade 2 or 3 with peritoneal gliomatosis underwent adjuvant chemotherapy. During follow up period, 6 mature teratomas recurred at sacrococcygeal area (n = 1) and contralateral ovary (n = 5). Five patients of them underwent reoperation and diagnosed as a mature teratoma, but one who had underwent a left salpingooophrectomy with right ovary cystectomy at initial operation was observed carefully. Teratomas were dominant in female patients and developed at various organs. Complete excision was needed for good prognosis. In case of immature teratoma, complete excision and appropriate chemotherapy according to grading can contribute to favorable results.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.2
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• pp.86-90
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• 2017

Hydrocephalus is characterized by accumulation of cerebrospinal fluid in the ventricles of the brain causing progressive ventricular dilatation. Accumulation of cerebrospinal fluid typically causes increased pressure inside the skull, this may result in headaches, vomiting, nausea, seizures, sleepiness and mental impairment. Patients with hydrocephalus may have dental manifestations such as changes in the occlusion and greater accumulation of plaque. The prevalence of dental caries is high due to the difficulty of oral care. The purpose of this case report is to describe the dental care for an uncooperative child with hydrocephalus under general anesthesia.

• Journal of Genetic Medicine
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• v.10 no.2
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• pp.113-116
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• 2013

Alexander disease is a rare degenerative leukodystrophy caused by dominant mutations in glial fibrillary acidic protein (GFAP). The neonatal form of Alexander disease may manifest as frequent and intractable seizures or obstructive hydrocephalus, with rapid progression leading to severe disability or death within two years. We report a case of a 50-day-old male who presented with intractable seizures and obstructive hydrocephalus. His initial magnetic resonance imaging (MRI) suggested a tumor-like lesion in the tectal area causing obstructive hydrocephalus. Despite endoscopic third ventriculostomy and multiple administrations of antiepileptic drugs, the patient experienced intractable seizures with rapid deterioration of his clinical status. After reviewing serial brain MRI scans, Alexander disease was suspected. Subsequently, we confirmed the de novo missense mutation in GFAP (c.1096T>C, Y366H). Although the onset was slightly delayed from the neonatal period (50 days old), we concluded that the overall clinical features were consistent with the neonatal form of Alexander disease. Furthermore, we also suspected that a Y366 residue might be closely linked to the neonatal form of Alexander disease based on a literature review.

• Journal of radiological science and technology
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• v.34 no.1
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• pp.59-64
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• 2011

We investigated the radiation exposure caused by DIPS, which is used to identify accurate repositioning and tumor location in pediatric cancer patients proton therapy. To compare and analyze DIPS condition, 50 pediatric cancer patients who underwent proton therapy were selected in Ilsan K cancer-specialized hospital from March 2007 to October 2009. For DIP exposure, 0.09~1.57 mGy is measured in AP and lateral directions and 23.55 mGy is measured in CSI patients. In whole brain patient, the amount of a day DIP exposure dose was 1.13 mGy. During treatment period, who exposed the biggest DIP dose are whole brain patients, 632.71 mGy is exposed. It is 1.13% of prescribed dose, represented dose is adequate because it is not exceeded 2% of recommended dose. Even though the exposed dose is not exceeded more than 2% of prescribed in DIP exposure, we should recognize the radiation damage and genetic influences to pediatric cancer patients, who is much sensitive to radiation and has longer mean residual life time. Therefore, DIPS guideline for pediatric cancer patients should be indicated to minimize the radiation exposure.

• Molecules and Cells
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• v.37 no.5
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• pp.357-364
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• 2014

Medulloblastoma, the most common malignant brain tumor in children, is a disease whose mechanisms are now beginning to be uncovered by high-throughput studies of somatic mutations, mRNA expression patterns, and epigenetic profiles of patient tumors. One emerging theme from studies that sequenced the tumor genomes of large cohorts of medulloblastoma patients is frequent mutation of RNA binding proteins. Proteins which bind multiple RNA targets can act as master regulators of gene expression at the post-transcriptional level to co-ordinate cellular processes and alter the phenotype of the cell. Identification of the target genes of RNA binding proteins may highlight essential pathways of medulloblastomagenesis that cannot be detected by study of transcriptomics alone. Furthermore, a subset of RNA binding proteins are attractive drug targets. For example, compounds that are under development as anti-viral targets due to their ability to inhibit RNA helicases could also be tested in novel approaches to medulloblastoma therapy by targeting key RNA binding proteins. In this review, we discuss a number of RNA binding proteins, including Musashi1 (MSI1), DEAD (Asp-Glu-Ala-Asp) box helicase 3 X-linked (DDX3X), DDX31, and cell division cycle and apoptosis regulator 1 (CCAR1), which play potentially critical roles in the growth and/or maintenance of medulloblastoma.