• Clinical and Experimental Pediatrics
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• 제60권5호
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• pp.129-137
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• 2017

The event-free survival (EFS) for pediatric acute lymphoblastic leukemia (ALL) has shown remarkable improvement in the past several decades. In Korea also, a recent study showed 10-year EFS of 78.5%. Much of the improved outcome for pediatric ALL stems from the accurate identification of prognostic factors, the designation of risk group based on these factors, and treatment of appropriate duration and intensity according to risk group, done within the setting of cooperative clinical trials. The schema of first-line therapy for ALL remains mostly unchanged, although many groups have now reported on the elimination of cranial irradiation in all patients with low rates of central nervous system relapse. Specific high risk subgroups, such as Philadelphia chromosome-positive (Ph+) ALL and infant ALL continue to have significantly lower survival than other ALL patients. The introduction of tyrosine kinase inhibitors into therapy has led to enhanced outcome for Ph+ ALL patients. Infant ALL patients, particularly those with MLL rearrangements, continue to have poor outcome, despite treatment intensification including allogeneic hematopoietic cell transplantation. Relapsed ALL is a leading cause of mortality in pediatric cancer. Recent advances in immunotherapy targeting the CD19 of the ALL blast have shown remarkable efficacy in some of these relapsed and refractory patients. With improved survival, much of the current focus is on decreasing the long-term toxicities of treatment.

• Journal of Korean Neurosurgical Society
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• 제61권3호
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• pp.319-332
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• 2018

Germline mutations in cancer causing genes result in high risk of developing cancer throughout life. These cancer predisposition syndromes (CPS) are especially prevalent in childhood brain tumors and impact both the patient's and other family members' survival. Knowledge of specific CPS may alter the management of the cancer, offer novel targeted therapies which may improve survival for these patients, and enables early detection of other malignancies. This review focuses on the role of CPS in pediatric high grade gliomas (PHGG), the deadliest group of childhood brain tumors. Genetic aspects and clinical features are depicted, allowing clinicians to identify and diagnose these syndromes. Challenges in the management of PHGG in the context of each CPS and the promise of innovative options of treatment and surveillance guidelines are discussed with the hope of improving outcome for individuals with these devastating syndromes.

• Asian Pacific Journal of Cancer Prevention
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• 제14권2호
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• pp.1101-1106
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• 2013

Background: Osteosarcoma is the most common primary bone tumor in childhood and adolescence. Carboplatin, a platinum-derived agent, is used as neoadjuvant chemotherapy for pediatric osteosarcoma because of its anti-tumor activity and had low toxicity as compared to cisplatin. Objective: To determine demographic data, prognostic factors and outcome of childhood osteosarcoma treated with a carboplatin-based chemotherapeutic protocol at Chiang Mai University. Method: A retrospective analysis was conducted on 34 osteosarcoma patients aged less than 18 years and treated between 2003 and 2011. Results: Overall limb-salvage and amputation rates were 23.5% and 70.6%, respectively. With the mean follow-up time of 29.5 months (1.5-108.9), the Kaplan-Meier analysis for 3-year disease-free survival (DFS) and 3-year overall survival (OS) were $20.2{\pm}7.7%$ and $47.1{\pm}9.5%$ respectively. Patients who had initial pulmonary metastasis were at significantly greater risk for developing recurrence (p=0.02, OR=7; 1.2-40.1) and had a tendency to have lower 3-year OS compared to those without initial pulmonary metastasis ($28.1{\pm}13%$, $63.1{\pm}12.3%$, respectively, p=0.202). On univariate analysis, age at diagnosis >14 years and patients who were declined surgery were significantly associated with lower 3-year OS (p=0.008 and <0.05, respectively). However, age at diagnosis, sex, tumor size and histological subtypes were not found to significantly affect recurrence or survival. Conclusions: In our study, the survival rate was far lower than those reported from developed countries. These might indicate the ineffectiveness of carboplatin in combination with doxorubicin as frontline treatment of pediatric osteosarcoma, especially in those with initial pulmonary metastasis. Refinement in risk and treatment stratification and dose intensification for pediatric osteosarcoma constitutes a future challenge to improve outcomes, especially in metastatic patients who may need a more intensive regimen.

• Journal of Radiation Protection and Research
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• 제46권3호
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• pp.98-105
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• 2021

Background: In recent events of the coronavirus disease 2019 (COVID-19) pandemic, computed tomography (CT) scans are being globally used as a complement to the reverse-transcription polymerase chain reaction (RT-PCR) tests. It will be important to be aware of major organ dose levels, which are more relevant quantity to derive potential long-term adverse effect, for Korean pediatric and adult patients undergoing CT for COVID-19. Materials and Methods: We calculated organ dose conversion coefficients for Korean pediatric and adult CT patients directly from Korean pediatric and adult computational phantoms combined with Monte Carlo radiation transport techniques. We then estimated major organ doses delivered to the Korean child and adult patients undergoing CT for COVID-19 combining the dose conversion coefficients and the international survey data. We also compared our Korean dose conversion coefficients with those from Caucasian reference pediatric and adult phantoms. Results and Discussion: Based on the dose conversion coefficients we established in this study and the international survey data of COVID-19-related CT scans, we found that Korean 7-year-old child and adult males may receive about 4-32 mGy and 3-21 mGy of lung dose, respectively. We learned that the lung dose conversion coefficient for the Korean child phantom was up to 1.5-fold greater than that for the Korean adult phantom. We also found no substantial difference in dose conversion coefficients between Korean and Caucasian phantoms. Conclusion: We estimated radiation dose delivered to the Korean child and adult phantoms undergoing COVID-19-related CT examinations. The dose conversion coefficients derived for different CT scan types can be also used universally for other dosimetry studies concerning Korean CT scans. We also confirmed that the Caucasian-based CT organ dose calculation tools may be used for the Korean population with reasonable accuracy.

• Advances in pediatric surgery
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• 제6권2호
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• pp.95-99
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• 2000

Thyroid carcinoma is relatively rare in children. Eight cases of thyroid carcinoma were among 18 patients operated upon for thyroid tumors at Pediatric Surgery, Asan Medical Center in 11 years' period between 1989 and 2000. Five patients were boys and three were girls. The age distribution ranged from 10 to 14 years with a median age of 11.6 years. We studied clinical presentations, diagnostic workup, pathology, treatment, and follow-up (recurrence, mortality, and survival). All patients presented with anterior neck mass but one with multiple cervical lymph nodes enlargement. Familial history of thyroid cancer was seen in one case. All patients had a cold nodule by 1-131 thyroid scan. Fine needle aspiration (FNA) biopsy was performed in all patients. The right lobe was involved in four patients and the left lobe in two. Two had both lobes involvement. Pathologically, six cases were papillary carcinoma, one was follicular carcinoma, and one insular carcinoma. Four patients underwent unilateral lobectomy and isthmectomy. Total thyroidectomy was performed in three patients. The insular carcinoma case was preoperatively diagnosed as follicular neoplasm by FNA. After confirmation of the pathology by initial right thyroid lobectomy, total thyroidectomy was subsequently done. Cervical lymph node metastases were presented in three cases (37.5%), and lung metastasis in two cases (25%). Three patients received postoperative I-131 ablation. After a median follow-up of 53 months, all patients were alive without evidence of recurrence. In conclusion, thyroid carcinoma in children is frequently associated with lymph nodes involvement and distant metastasis, however, the prognosis is relatively good.

• Clinical and Experimental Pediatrics
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• 제54권3호
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• pp.128-132
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• 2011

Purpose: Cisplatin-based chemotherapy has been commonly used for the treatment of intracranial germ cell tumors (IC-GCTs). However, this treatment exhibits some adverse effects such as renal problems and hearing difficulty. Carboplatin-based chemotherapy was administered to pediatric patients with IC-GCTs from August 2004 at the Samsung Medical Center. In this study, we assessed the responses and adverse effects of carboplatin-based chemotherapy in pediatric IC-GCTs patients according to the risk group, and compared the results with those of the previous cisplatin-based chemotherapy. Methods: We examined 35 patients (27 men and 8 women) diagnosed with IC-GCTs between August 2004 and April 2008 and received risk-adapted carboplatin-based chemotherapy at the Samsung Medical Center. Patients were divided into either low-risk (LR) or high-risk (HR) groups and a retrospective analysis was performed using information from the medical records. Results: Although hematological complications were common, hearing difficulties or grade 3 or 4 creatinine level elevation were not observed in patients who underwent carboplatin-based chemotherapy. The frequency of febrile neutropenia did not differ between the risk groups. The overall survival was 100% and event-free survival (EFS) was 95.7%. The EFS rate was 100% in the LR group and 90% in the HR group, respectively. Conclusion: Despite their common occurrence in high-risk patients, no lethal hematological complications were associated with carboplatin-based treatment. The current carboplatin-based chemotherapy protocol is safe and effective for the treatment of pediatric patients with IC-GCTs.

• Clinical and Experimental Pediatrics
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• 제56권2호
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• pp.60-67
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• 2013

As chemotherapy and other sophisticated treatment strategies evolve and the number of survivors of long-term childhood cancer grows, the long-term complications of treatment and the cancer itself are becoming ever more important. One of the most important but often neglected complications is osteoporosis and increased risk of fracture during and after cancer treatment. Acquisition of optimal peak bone mass and strength during childhood and adolescence is critical to preventing osteoporosis later in life. However, most childhood cancer patients have multiple risk factors for bone mineral loss. Cancer itself, malnutrition, decreased physical activity during treatment, chemotherapeutic agents such as steroids, and radiotherapy cause bone mineral deficit. Furthermore, complications such as growth hormone deficiency and musculoskeletal deformity have negative effects on bone metabolism. Low bone mineral density is associated with fractures, skeletal deformity, pain, and substantial financial burden not only for childhood cancer survivors but also for public health care systems. Thus, it is important to monitor bone health in these patients and minimize their risk of developing osteoporosis and fragility fractures later in life.

• Pediatric Infection and Vaccine
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• 제18권1호
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• pp.40-47
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• 2011

목 적 : 노로바이러스는 바이러스성 설사의 흔한 원인이며, 면역 저하 환자에서도 심각한 감염증을 유발하는 것으로 알려져 있어, 소아 암환자에서 노로바이러스 감염의 임상 양상을 알아보고자 하였다. 방 법: 2008년 11월부터 2009년 9월까지 삼성 서울병원에 입원한 환아 중 대변에서 노로바이러스 PCR 양성을 보였던 소아 암환자를 대상으로 하였고, 이들의 의무기록을 후향적으로 분석하였다. 결 과:연구에 포함된 환자는 총 10명이었으며, 이들 연령의 중앙값은 0.83세, 남녀의 비율은 1.5대 1이었다. 기저질환은 혈액암 4명, 신경모세포종 4명, 뇌종양 2명이었고, 감염 진단 당시 조혈모세포이식 수여자는 4명이었다. 모든 환자에서 설사 증상을 보였으며, 설사 횟수의 중앙값은 8.5회/일, 바이러스 배출 기간의 중앙값은 72.5일이었다. 장기종 소견을 보인 4명의 환자는 금식 및 총정맥 영양을 투여 받았으며, 노로바이러스 관련 사망은 관찰되지 않았다. 결 론 : 노로바이러스 감염은 소아 암환자에서 다양한 임상 증상을 유발 할 수 있으며, 이들 면역저하 환자에서 바이러스 배출 기간이 매우 길어, 설사가 있는 암환자에서 고려해야 할 바이러스 감염이다.

• Asian Pacific Journal of Cancer Prevention
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• 제14권7호
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• pp.4359-4363
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• 2013

Background: Pediatric lymphadenopathy is a challenging medical situation for the child patient, the parents, and the physician. Although the bulk of masses will be benign the fear of malignancy is omnipresent. Therefore, the objective of this study was to identify the common cytopathological patterns of lymphadenopathy among Sudanese children. Methods: One hundred pediatric patients presenting with peripheral lymphadenopathy were included in the study, their ages ranging from 2 to 14 years, with a mean age of 7 years. Demographic characteristics, clinical manifestations and FNA materials were prospectively obtained. Results: FNA was performed in 100 cases (100%). There were no technical complications. All cases confirmed adequacy of specimen. Overall, FNA demonstrated 90 (90%) benign lesions and 10 (10%) malignant diagnosis. The benign lesions were reactive lymphoid hyperplasia (n=64), followed by benign granulomatous disease (n=26). Of the 10 cases diagnosed with malignancy, 7 (7%) were cases of non-Hodgkin`s lymphoma and the remaining 3 (3%) were Hodgkin's lymphomas. Conclusion: Pediatric lymphadenopathy is common in Sudan. CLA is the common frequent site. Lymphoma represents a major challenge in this setting.

• Asian Pacific Journal of Cancer Prevention
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• 제16권17호
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• pp.7523-7527
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• 2015

Background: Pediatric acute lymphoblastic leukemia (pALL) patients at King Abdulaziz Medical City represent a pure Saudi Arabian population. ETV6-RUNX1 positive pALL patients have good prognosis as compared to ETV6-RUNX1 negative counterparts. Therefore, frequencies of these two patient groups have a huge consideration in treatment strategies of pALL in a given population. Different geographical locations have been reported to have different frequencies of ETV6-RUNX1 ranging from 10% in Southeast Asia to 30% in Australia. Aim: Therefore, the objective of this study was to establish the ETV6-RUNX1 status of Saudi Arabian pALL patients and its association with clinical parameters and early remission. Materials and Methods: Clinical parameters and ETV6-RUNX1 status (using FISH technique) of pALL patients attending the Pediatric Oncology Clinic, King Abdulaziz Medical City, Riyadh from 2006 to 2011 were studied. Comparisons between ETV6-RUNX1 positive and negative groups were accomplished using chi-square test or Fisher's exact test. All statistical analyses were performed using SAS version 9.2 (SAS Institute, Inc., Cary, NC). Results: Out of 54 patients, 33 were male and 21 were females (ratio 1.57:1). B- and T-cell lineages were found in 47 (87%) and 7 (13%) patients respectively. Only 5 (9.3%) patients were ETV6-RUNX1 positive while 49(80.7%) were ETV6-RUNX1 negative. All ETV6-RUNX1 patients (100%) were of B-cell lineage and 80% (4/5) were in the 3-7 year age group. None of the ETV6-RUNX11 patients had ${\geq}5%$ blasts (no remission) at day 14 as compared with 9% in the ETV6-RUNX1 negative group (Figure 1). Conclusions: Frequency of ETV6-RUNX1 positive patients (less than 10%) in our pALL patients is much lower than reported for most European countries, North America, Australia and Japan while it is in accordance with ETV6-RUNX1 frequencies from Egypt (11.6%), Pakistan (10%), Spain (2%) and India (5-7%). This shows ethnic differences in genetics of pALL as well as higher frequencies of ETV6-RUNX1 positive pALL mostly in more industrialized countries, probably due to some industrial pollutants or westernized lifestyle.