• 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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• 제49권1호
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• pp.76-81
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• 2006

목 적 : 뇌종양에 의한 임상증상은 뇌종양 발생의 발생위치와 밀접한 관계를 가지는 것으로 알려져 있다. 본 연구에서는 소아 뇌종양 환자에서 발생위치에 따라 증상의 차이와 증상 발현시부터 보호자와 의료진이 종양을 의심하고 진단하기까지 걸리는 시간의 차이에 대해 조사하였다. 방 법 : 1996년 7월부터 2004년 6월까지 인하대학교병원 소아과에서 뇌종양으로 진단받은 15세 이하의 환아를 대상으로 종양의 위치와 증상, 보호자와 의료진이 증상이 나타나기부터 진단을 하기까지 얼마의 시간이 경과했는지에 관해 후향적 고찰을 시행하였다. 발생위치는 방사선검사상(자기공명영상)에서 나타난 위치에 따른 진단을 근거로 분류하였다. 결 과 : 뇌종양으로 진단 받은 환아는 모두 45명이었고 남아가 27명, 여아가 18명이었다. 대상 환아에서 발병연령의 중앙값은 천막상부는 6.0세, 천막하부는 7.0세로 두 군간의 유의한 차이는 없었다. 발생위치는 천막상부에 24명(53.3%), 천막하부에 21명(46.6%) 발생하였고 천막상부 종양에서는 대뇌반구 및 측뇌실 종양이 14명(58.3%), 터키안 상부 종양이 7명(29.1%), 송과체 및 제3 뇌실 후반부종양이 3명(12.5%)을 각각 차지하였으며 천막하부 종양에서는 소뇌충부 및 제4 뇌실종양이 12명(57.1%), 소뇌 반구종양이 4명(19.1%), 뇌간 종양이 5명(23.8%)을 차지하였다. 종양으로 진단시 환아들의 주증상은 천막상부 종양에서는 경련이 9명(37.5%)으로 가장 많았고 두통은 3명(12.5%)을 차지한 반면 천막하부 종양에서는 두통이 8명(38.0%)으로 가장 많았고 경련이 1명(4.8%)으로 조사되어 천막상부 종양에서는 경련이 천막하부에 비하여 많이 나타났고(P=0.012) 천막하부 종양에서는 두통이 천막상부에 비하여 많은 것으로 나타났다(P=0.046). 이밖에도 천막상부 종양에서는 성장지연(2명), 성조숙증(2명) 등이, 천막하부 종양에서는 운동실조(3명), 구토(3명) 등이 우세하게 나타났으나 통계학적으로 유의성은 없었다. 보호자가 증상 인식 후 병원을 내원하기까지 기간은 천막상부는 중앙값이 6일(즉시-8개월), 천막 하부는 중앙값이 30일(즉시-2개월)로 나타났으나 두 군간의 유의한 차이는 없었다(P=0.088). 병원에 내원 후 의료진이 진단하기까지 기간은 천막상부와 하부에서 모두 내원 즉시 의심을 하여 방사선검사로 확진된 경우가 대부분 이었으나 천막상부의 경우 6년, 하부의 경우 7개월까지 지연된 예가 있었다. 결 론 : 소아에서 뇌종양이 진단될 때 천막상부 종양에서는 경련이 천막하부 종양에서는 두통이 각각 가장 많은 비중을 차지하였다. 보호자가 증상을 발견하고 내원하는 기간과 병원에 내원 후 종양이 진단되기까지 기간은 오래 걸리지 않는 경향을 보였지만, 두통이나 경련으로 내원하는 소아환자에서 자세한 병력청취와 신경학적 검사 및 두개 방사선 영상검사를 통해 뇌종양을 의심하는 것이 중요하다.

• Journal of Korean Neurosurgical Society
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• 제61권3호
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• pp.352-362
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• 2018

Gliomas are the most common pediatric tumors of the central nervous system. In this review, we discuss the clinical features, treatment paradigms, and evolving concepts related to two types of pediatric gliomas affecting two main locations: the optic pathway and thalamus. In particular, we discuss recently revised pathologic classification, which adopting molecular parameter. We believe that our review contribute to the readers' better understanding of pediatric glioma because pediatric glioma differs in many ways from adult glioma according to the newest advances in molecular characterization of this tumor. A better understanding of current and evolving issues in pediatric glioma is needed to ensure effective management decision.

• 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.

• 대한장애인치과학회지
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• 제15권1호
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• pp.74-78
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• 2019

AT/RT를 갖는 환자는 빈번하게 MRI촬영을 시행하게 된다. MRI의 경우 강자성 효과(ferromagnetic effect)로 인해 스테인레스스틸 기성금속관의 탈락이 발생할 위험성이 있고 영상의 인공물(artifact)의 형성을 유발할 수 있으므로 유치의 전장관 수복 전에 재료의 선택에 있어서 이러한 위험성에 대해 고려해야 한다. 이 경우 기성 지르코니아 전장관은 AT/RT 환자들의 크라운 수복에 있어서 좋은 수복재료로 사용될 수 있다. 또한 항암치료로 인해 AT/RT 환아들은 우식발생율이 높으므로 구강위생 관리 및 전문가 불소도포 등의 주기적인 정기검진이 요구된다.

• Journal of Korean Medical Science
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• 제33권51호
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• pp.329.1-329.9
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• 2018

Background: Brain magnetic resonance imaging (MRI) is routinely performed to identify brain lesions in girls with central precocious puberty (CPP). We aimed to investigate the prevalence and type of brain lesions among Korean girls with CPP and evaluate the need for routine brain MRI examinations. Methods: This retrospective cross-sectional study evaluated data on 3,528 girls diagnosed with CPP from April 2003 to December 2016, and identified 317 girls who underwent sellar MRI. Exclusion criteria were patients with a known brain tumor or who did not undergo brain MRI due to refusal or the decision of the pediatric endocrinologist. Results: Normal sellar MRI findings were observed in 291 of the 317 girls (91.8%). Incidental findings were observed in 26 girls (8.2%). None of the patients had pathological brain lesions. Conclusion: The prevalence of intracranial lesions among girls who were generally healthy and without neurological symptoms but diagnosed with CPP was lower than that previously reported. Furthermore, none of the identified lesions required treatment. It may be prudent to reconsider the routine use of brain MRI to screen all patients with CPP, especially if they are healthy and neurologically asymptomatic, and are girls aged 6-8 years.

• 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.

• Asian Pacific Journal of Cancer Prevention
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• 제16권13호
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• pp.5149-5152
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• 2015

Cancer-testis antigens (CTAs) are a group of tumor-associated antigens with more than 140 members whose expression has been shown to be limited to gametogenic tissues and placenta among normal tissues. However, malignant tissues of different origins have shown aberrant and elevated expression of these antigens. Such a pattern of expression endows beneficial properties for use as cancer biomarkers as well as immunotherapeutic targets as a result of the immune-privileged status of the testes. CTAs have been shown to be expressed in pediatric brain tumors, different types of sarcomas, leukemias, and lymphomas as well as neuroblastomas. Although data regarding their expression pattern in childhood tumors are not as comprehensive as for adult tumors, it is supposed that CTA-based immunotherapeutic approaches can also be used for pediatric cancers. However, there are limited data about the objective clinical responses following immunotherapy in such patients. Here we try to review the available information.

• Advances in pediatric surgery
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• 제15권1호
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• pp.27-37
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• 2009

Neuroblastoma is the most common extracranial solid tumor in children. We retrospectively analyzed the results of neuroblastoma treatment of 191 patients (116 males and 75 females) treated between January 1986 and December 2005 at the Department of Pediatric Surgery and the Department of Pediatrics, Seoul National University Children's Hospital. The mean age at diagnosis was 3.1 years (0.1 yrs - 13.5 yrs). Forty-seven patients were under 1 year of age. The mean follow-up period was 57.3 months (24 days - 19.1 yrs). Patients were classified into two groups according to the completeness of resection of the primary tumor; (1) gross total resection (GTR) and (2) incomplete resection (IR). The number of patients in stages I, II, III, IV, IV-S were 17 (8.9 %), 12 (6.3 %), 43 (22.5 %), 114 (59.7 %), 4 (2.1 %), respectively. GTR was achieved in 120 patients and IR in 71 (22 stage III, 47 stage IV, 1 stage IV-S, 1 brain). Overall survival (OS) was 65.2 % and event-free survival (EFS) was 48.6 %. EFS were 100 %, 75 %, 66.8 %, 31.3 %, 75 % at stage I, II, III, IV, IV-S, respectively. There was no significant difference in EFS according to the completeness of resection. EFS was improved in GTR group (p=ns) of stage III, but by contrast, stage IV patients showed worse EFS in GTR group. EFS was improved significantly after the introduction of autologous stem cell transplantation (ASCT) (58.1% vs. 40.6%, p=.029). The EFS improved significantly after the introduction of ASCT in IR group (p=.009) rather than GTR group (p=ns). The EFS of the patients under 1 year of age (N=47) was better than the patients over 1 year of age (N=144) significantly (75.5 % vs. 39.4 %, p=.0034). The prognosis of neuroblastoma was related to the INSS stage and age at diagnosis. The survival of IR group significantly improved after ASCT.

• 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.