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Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia in Childhood
구홍회 대한소아청소년과학회 2011 Clinical and Experimental Pediatrics (CEP) Vol.54 No.3
In pediatric patients with acute lymphoblastic leukemia (ALL), the Philadelphia chromosome translocation is uncommon, with a frequency of less than 5%. However, it is classified as a high or very high risk, and only 20‒30% of Philadelphia chromosome-positive (Ph+)children with ALL are cured with chemotherapy alone. Allogeneic hematopoietic stem cell transplantation from a closely matched donor cures 60% of patients in first complete remission. Recent data suggest that chemotherapy plus tyrosine kinase inhibitors (TKIs) may be the initial treatment of choice for Ph+ ALL in children. However, longer observation is required to determine whether long-term outcome with intensive imatinib and chemotherapy is indeed equivalent to that with allogeneic related or alternative donor hematopoietic stem cell transplantation (HSCT). Reports on the use of second-generation TKIs in children with Ph+ ALL are limited. A few case reports have indicated the feasibility and clinical benefit of using dasatinib as salvage therapy enabling HSCT. However, more extensive data from clinical trials are needed to determine whether the administration of secondgeneration TKIs in children is comparable to that in adults. Because Ph+ ALL is rare in children, the question of whether HSCT could be a dispensable part of their therapy may not be answered for some time. An international multicenter study is needed to answer the question of whether imatinib plus chemotherapy could replace sibling allogeneic HSCT in children with Ph+ ALL.
구홍회 대한소아청소년과학회 2007 Clinical and Experimental Pediatrics (CEP) Vol.50 No.7
Since the introduction of chemotherapy for the treatment of childhood leukemia more than 50 years ago, the results of childhood cancer have improved dramatically. The 5-year survival rate of disease, many of which were uniformly fatal in the prechemotherapy era, reached to more than 75%. This remarkable improvement in survival is a direct result of the incorporation of chemotherapeutics into treatment regimens that previously relied only on surgery or radiotherapy for the primary tumor. The multimodality approach, which integrates surgery and radiotherapy to control local disease with chemotherapy to eradicate systemic or metastatic disease, has become the standard approach to treating most childhood cancers. The overall improvement in outcomes in childhood solid tumors has been related to the development of multidisplinary cooperative studies that has permitted the development of well-designed tumor treatment protocols characterized by uniform staging criteria, sharing informations in pathologic classification, uniform methods for tumor markers, oncogenes, and other biologic and genetic factors. Important advances in the biologic study of cancer and its genetic basis led to a number of observations that impact directly on the management of childhood solid tumors. Identification of specific genes, oncogenes, tumor markers, and other biologic and pathologic factors plays an important role in both staging and clarifying the risk categorization of individual patients. Treatment of the patient is influenced by the recognition of specific risk factors. This knowledge has resulted in a change in the approach to care based not only on staging criteria, but also on risk-based management. This concept uses various risk factors of outcomes. Risk-based management allows for each patient to maximize survival, minimize long-term morbidity and improve the quality of life, especially for children's growth and development.
흉막폐아세포종(Pleuropulmonary Blastoma) 치험 2예 보고
박준석,한정호,구홍회,김진국 대한흉부외과학회 2003 Journal of Chest Surgery (J Chest Surg) Vol.36 No.8
흉막폐아세포종은 소아에 국한하여 생기는 매우 드문 원발성 악성종양이며 극히 나쁜 예후를 보인다. 주 증상은 흉부불쾌감, 호흡곤란, 반복적인 상기도 감염, 발열, 마른 기침, 그리고 흉통 등이다. 흉막폐아세포종은 매우 빠른 진행양상을 보이며, 폐문 및 종격 림프절에 전이될 수 있다. 원격전이는 뇌, 골조직, 그리고 복강 내 장기들에서 보인다. 흉막폐아세포종의 치료는 다각적 접근을 요한다. 수술에 의한 종괴의 일차적 제거가 우선적인 치료법이나, 종양의 크기나 침범 범위로 인해 일차적으로 수술적 제거가 힘든 경우 수술 전 신보조항암요법으로 종양의 크기를 줄일 수 있으며, 이후 수술적인 완전절제를 고려할 수 있다. 본원에서는 소아에서 발생한 흉막폐아세포종에 대해 신보조항암요법, 수술적 절제 및 보조항암요법을 통해 성공적으로 치료한 2예를 경험하고 이를 보고하고자 한다.
Desmoplastic small round cell tumor of the stomach mimicking a gastric cancer in a child
문석배,허정민,구홍회,서연림,신현백,서정민,이석구 대한외과학회 2011 Annals of Surgical Treatment and Research(ASRT) Vol.80 No.6
Intra-abdominal desmoplastic small round cell tumor (DSRCT) is a highly malignant tumor of uncertain histogenesis. Here we report a case of DSRCT involving the stomach, initially misdiagnosed as gastric cancer. A 12-year-old boy presented with upper abdominal pain developed 1 month prior. On gastroscopy, a 7-cm mass was noted involving the esophago-gastric junction to the fundus, and positron emission tomography showed multiple hot uptakes suggesting distant metastasis. Gastroscopic biopsy showed poorly differentiated malignant cells. We diagnosed as stage IV gastric cancer and treated with 6 cycles of chemotherapy. Laparotomy revealed a huge gastric mass along with peritoneal disseminations. Palliative proximal gastrectomy was performed. Pathological examination revealed transmural involvement of DSRCT, and t(11;22)(p12;q12) was demonstrated on fluorescence in situ hybridization test. The chemotherapeutic regimen was changed and the patient underwent 8 additional cycles of post-operative chemotherapy. The patient is now alive and the residual tumor shows no significant changes after chemotherapy.
Suntae Ji,구홍회,Hee Won Chueh,Ju Youn Kim,임수진,Eun Joo Cho,Soo Hyun Lee,유건희,성기웅 대한소아청소년과학회 2011 Clinical and Experimental Pediatrics (CEP) Vol.54 No.3
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 riskadapted 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 carboplatinbased treatment. The current carboplatin-based chemotherapy protocol is safe and effective for the treatment of pediatric patients with IC-GCTs.