• Journal of Korean Neurosurgical Society
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• 제30권sup1호
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• pp.140-143
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• 2001

We report a case of huge trigeminal schwannoma in a 10-year-old boy with neurofibromatosis type II, extending into the three spaces of the left middle, posterior, and infratemporal fossa. Initially we thought the child had a solitary trigeminal schwannoma, and most of the mass was resected successfully through one-stage operation, cranio-orbito-zygomatic intradural approach. However during the follow-up of the patient we found radiographically other multiple intracranial tumors of bilateral acoustic schwannomas, right trigeminal schwannoma, and foramen magnum tumor. Eventually the patient was diagnosed as neurofibromatosis type II presenting multiple intracranial tumors. We think childhood trigeminal schwannoma, even though in the case of solitary tumor, should be considered as possible initial manifestation of neurofibromatosis type II and that careful follow-up for the possibility of occurrence of other brain tumors such as schwannomas or meningiomas is necessary.

• Journal of Korean Neurosurgical Society
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• 제49권3호
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• pp.147-152
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• 2011

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 ${\alpha}$ 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.

• Clinical and Experimental Pediatrics
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• 제57권7호
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• pp.333-336
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• 2014

Reports of constitutional ring chromosome 22, r(22) are rare. Individuals with r(22) present similar features as those with the 22q13 deletion syndrome. The instability in the ring chromosome contributes to the development of variable phenotypes. Central nervous system (CNS) atypical teratoid rhabdoid tumors (ATRTs) are rare, highly malignant tumors, primarily occurring in young children below 3 years of age. The majority of ATRT cases display genetic alterations of SMARCB1 (INI1/hSNF5 ), a tumor suppressor gene located on 22q11.2. The coexistence of a CNS ATRT in a child with a r(22) is rare. We present a case of a 4-month-old boy with 46,XY,r(22)(p13q13.3), generalized hypotonia and delayed development. High-resolution microarray analysis revealed a 3.5-Mb deletion at 22q13.31q13.33. At 11 months, the patient had an ATRT ($5.6cm{\times}5.0cm{\times}7.6cm$) in the cerebellar vermis, which was detected in the brain via magnetic resonance imaging.

• Journal of Korean Neurosurgical Society
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• 제61권3호
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• pp.393-401
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• 2018

The prognosis of brain tumors in children has improved for last a few decades. However, the prognosis remains dismal in patients with recurrent brain tumors. The outcome for infants and young children in whom the use of radiotherapy (RT) is very limited because of unacceptable long-term adverse effect of RT remains poor. The prognosis is also not satisfactory when a large residual tumor remains after surgery or when leptomeningeal seeding is present at diagnosis. In this context, a strategy using high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT) has been explored to improve the prognosis of recurrent or high-risk brain tumors. This strategy is based on the hypothesis that chemotherapy dose escalation might result in improvement in survival rates. Recently, the efficacy of tandem HDCT/auto-SCT has been evaluated in further improving the outcome. This strategy is based on the hypothesis that further dose escalation might result in further improvement in survival rates. At present, the number of studies employing tandem HDCT/auto-SCT for brain tumors is limited. However, results of these pilot studies suggest that tandem HDCT/auto-SCT may further improve the outcome. In this review, we will summarize our single center experience with tandem HDCT/auto-SCT for recurrent or high-risk brain tumors.

• Journal of Korean Neurosurgical Society
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• 제61권3호
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• pp.363-375
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• 2018

Intraoperative monitoring (IOM) utilizes electrophysiological techniques as a surrogate test and evaluation of nervous function while a patient is under general anesthesia. They are increasingly used for procedures, both surgical and endovascular, to avoid injury during an operation, examine neurological tissue to guide the surgery, or to test electrophysiological function to allow for more complete resection or corrections. The application of IOM during pediatric brain tumor resections encompasses a unique set of technical issues. First, obtaining stable and reliable responses in children of different ages requires detailed understanding of normal age-adjusted brain-spine development. Neurophysiology, anatomy, and anthropometry of children are different from those of adults. Second, monitoring of the brain may include risk to eloquent functions and cranial nerve functions that are difficult with the usual neurophysiological techniques. Third, interpretation of signal change requires unique sets of normative values specific for children of that age. Fourth, tumor resection involves multiple considerations including defining tumor type, size, location, pathophysiology that might require maximal removal of lesion or minimal intervention. IOM techniques can be divided into monitoring and mapping. Mapping involves identification of specific neural structures to avoid or minimize injury. Monitoring is continuous acquisition of neural signals to determine the integrity of the full longitudinal path of the neural system of interest. Motor evoked potentials and somatosensory evoked potentials are representative methodologies for monitoring. Free-running electromyography is also used to monitor irritation or damage to the motor nerves in the lower motor neuron level : cranial nerves, roots, and peripheral nerves. For the surgery of infratentorial tumors, in addition to free-running electromyography of the bulbar muscles, brainstem auditory evoked potentials or corticobulbar motor evoked potentials could be combined to prevent injury of the cranial nerves or nucleus. IOM for cerebral tumors can adopt direct cortical stimulation or direct subcortical stimulation to map the corticospinal pathways in the vicinity of lesion. IOM is a diagnostic as well as interventional tool for neurosurgery. To prove clinical evidence of it is not simple. Randomized controlled prospective studies may not be possible due to ethical reasons. However, prospective longitudinal studies confirming prognostic value of IOM are available. Furthermore, oncological outcome has also been shown to be superior in some brain tumors, with IOM. New methodologies of IOM are being developed and clinically applied. This review establishes a composite view of techniques used today, noting differences between adult and pediatric monitoring.

• Child Health Nursing Research
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• 제9권1호
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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.

• 대한한방소아과학회지
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• 제12권1호
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• pp.55-61
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• 1998

Brainstem gliomas remain an important oncologic problem in the pediatric age group. These tumors constitute between 10 and 15% of all intracranial childhood tumors and despite advanced in the diagnosis and treatment of children with brain tumors, brainstem gliomas are still almost invariably rapidly lethal. We have few of clinical records of braintumors and admission case in the oriental medical hospital, classifications of tumor, symptoms and etc, specially in the pediatrics, so we introduce a case of a 7-year-old child with brainstem glioma which is diagnosised by MRI in our hospital.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제6권2호
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• pp.215-220
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• 2003

저자들은 생후 3개월부터 심한 성장장애를 보였던 남아가 14개월에 두 개 내 종양에 의해 발생한 간뇌증후군으로 진단되었기에 보고하는 바이다. 소아에서 적당한 양을 섭취하고 식욕이 유지됨에도 불구하고 현저한 성장장애와 체중 증가 부진을 보이는 경우에는 간뇌증후군의 감별이 필요할 것으로 사료된다.

• 한국간담췌외과학회지
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• 제28권1호
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• pp.34-41
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• 2024

Backgrounds/Aims: Liver transplantation (LT) provides a favorable outcome for patients with hepatocellular carcinoma (HCC) and was launched in Vietnam in 2004. In this study, we evaluated the short-term and long-term outcomes of LT and its risk factors. Methods: This retrospective study analyzed HCC patients who underwent LT at Viet Duc University hospital, Vietnam, from 01/2012-03/2022. The following data were gathered: demographics, virus infection, tumor characteristics, alpha-fetoprotein (AFP) level, Child-Pugh and MELD scores, selection criteria, type of LT, complications, 30-day mortality, and disease-free and overall survival (DFS and OS). Results: Fifty four patients were included, the mean age was 55.39 ± 8.46 years. Nearly 90% had hepatitis B virus-related HCC. The median (interquartile range) AFP level was 16.2 (88.7) ng/mL. The average MELD score was 10.57 ± 5.95; the rate of Child-Pugh A and B were 70.4% and 18.5%, respectively. Nearly 40% of the patients were within Milan criteria, brain-dead donor was 83.3%. Hepatic and portal vein thrombosis occurred in 0% and 1.9%, respectively; hepatic artery thrombosis 1.9%, biliary leakage 5.6%, and postoperative hemorrhage 3.7%. Ninety-day mortality was 5.6%. Five-year DFS and OS were 79.3% and 81.4%, respectively. MELD score and ChildPugh score were predictive factors for DFS and OS (p < 0.05). In multivariate analysis, Child-Pugh score was the only significant factor (p < 0.05). Conclusions: In Vietnam, LT is an effective therapy for HCC with an acceptable complication rate, mortality rate, and good survival outcomes, and should be further encouraged.

• Journal of Korean Neurosurgical Society
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• 제61권3호
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• pp.386-392
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• 2018

Radiation therapy is highly effective for the management of pediatric malignant central nervous system (CNS) tumors including embryonal tumors. With the increment of long-term survivors from malignant CNS tumors, the radiation-related toxicities have become a major concern and we need to improve the treatment strategies to reduce the late complications without compromising the treatment outcomes. One of such strategies is to reduce the radiation dose to craniospinal axis or radiation volume and to avoid or defer radiation therapy until after the age of three. Another strategy is using particle beam therapy such as proton beams instead of photon beams. Proton beams have distinct physiologic advantages over photon beams and greater precision in radiation delivery to the tumor while preserving the surrounding healthy tissues. In this review, I provide the treatment principles of pediatric CNS embryonal tumors and the strategic improvements of radiation therapy to reduce treatment-related late toxicities, and finally introduce the increasing availability of proton beam therapy for pediatric CNS embryonal tumors compared with photon beam therapy.