• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1534-1539
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• 2002

Purpose : This study was designed to exclude radiation in advanced(stage 3, 4) Wilms tumor (WT) by increasing the chance of complete surgical removal with preceding neoadjuvant chemotherapy, thereby reducing the incidence of late effects. Methods : Between December 1998 and July 2002, we conducted neoadjuvant chemotherapy after needle aspiration biopsy on patients who had advanced WT. If needle biopsy was accessible, we conducted neoadjuvant chemotherapy(vincristine, adriamycin, dactinomycin) for 12 weeks and then performed surgical removal, excluded radiation therapy and conducted postoperative chemotherapy (vincristine, dactinomycin${\pm}$adriamycin). In other cases, we firstly conducted the operation and then performed radiation and postoperative chemotherapy. Results : Of the 17 patients diagnosed as WT, 12 patients had an advanced stage of disease. In two of the 12 patients, initial surgical removal was conducted. The median age of patients was 21 months(5-103 months). Of the 10 the patients who received neoadjuvant chemotherapy, eight patients were stage 1, one patient was stage 2, and the other was stage 3 at operation. In nine patients except one with stage 3 disease, we could perform complete surgical resection and therefore could omit radiation. In four cases we could also exclude adriamycin after operation. All but one patient was alive, disease-free, for a median follow-up of 21 months(9-43 months). Conclusion : After neoadjuvant chemotherapy, we could increase the chance of complete tumor resection, exclude radiation and decrease the intensity of postoperative chemotherapy in selected cases. Long term follow-up is needed to determine whether our method would significantly decrease late effects.

• Journal of Life Science
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• v.21 no.8
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• pp.1067-1075
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• 2011

Coiled-coil domain-containing protein 98 (CCDC98) plays a role in G2/M DNA damage checkpoint pathways by recruiting breast cancer 1 (BRCA1)-A complex to the DNA-damaged sites. However, the molecular mechanism of CCDC98 on the DNA damage-induced G2/M checkpoint pathways is unclear. In this study, we identifed Wilms tumor 1-associating protein (WTAP) as a novel CCDC98-binding protein, using tandem affinity purification. We confirmed the association between CCDC98 and WTAP using in vivo and in vitro binding assays. We demonstrated that CCDC98 regulates cyclin B1 expression by affecting WTAP protein stability. Based on these results, we suggest that CCDC98 may act as a novel cell cycle regulator by regulating the expression level of cyclin B1.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.16
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• pp.7303-7307
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• 2015

Background: Leukemia is a common cancer among children and adolescents. Wilms' tumor gene (WT1) is highly expressed in patients with acute leukemia. It is found as a tumor associated antigen (TAA) in various types of hematopoietic malignancies and can be employed as a useful marker for targeted immunotherapy and monitoring of minimal residual disease (MRD). In this regard, WT1 is a transcription factor that promotes gene activation or repression depending on cellular and promoter context. The purpose of this study was assessment of WT1 gene expression in patients with acute leukemia, measurement of IL-12 and C3 levels in serum and evaluation of the relationship between them. Materials and Methods: We evaluated the expression of WT1 mRNA using real-time quantitative RT-PCR and serum levels of IL-12 and C3 using ELISA and nephelometry in peripheral blood of 12 newly diagnosed patients with acute leukemia and 12 controls. Results: The results of our study showed that the average wT1 gene expression in patients was 7.7 times higher than in healthy controls (P <0.05). In addition, IL-12 (P = 0.003) and C3 (P <0.0001) were significantly decreased in the test group compared to controls. Conclusions: WT1 expression levels are significantly higher in patients compared with control subjects whereas serum levels of interleukin-12 and C3 are significantly lower in patients. Wt1 expression levels in patients are inversely related with serum levels of IL-12 and C3.

• Water for future
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• v.7 no.2
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• pp.75-82
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• 1974

본시험은 관개 및 배수 설계를 위한 기초자료 제공을 목적으로 토양수분 및 지하수위의 변화상태를 구명하고자 하는 것으로 카나다 퀴백(Quebec) 지방 저지대의 대표적인 2종의 태양인 점토와 사질로움 토양에 대해서 일차적으로 지표, 지하배수간의 토양수분과 지하수위의 변화를 연구분석한 것으로 그 결과를 요약하면 다음과 같다. 1. 2종의 토양(점토, 사질로움)에 있어서 공히 토양수분은 지표로부터 지하로 내려갈수록 증가되는 상태를 나타냈으면 이는 하층토로 내려감에 따라 팽창된 치밀한 토양 조직을 가지고 있음을 시사하며 2. 동기에 있어서는 지표로부터 12inch 깊이의 토양수분이 더 깊은 18inch나 24inch 깊이의 토양수분보다 증가된 상태를 유지하였다. 이는 토양내의 빙결막이나 지표면의 눈 또는 어름에 영향으로 생각되며 이 경향은 점토질 토양에서 보다 토양내 빙결막으로의 수분 이종을 조장해주는 투수 계수가 큰 사질로움 토양에서 더 높았다. 3. 양식험구 공히 지표에서 얕은 지하 0∼3inch 이내의 토양수분은 항우를 전후해서 급격한 변화를 가져왔다. 여기에서 수분의 급상승은 호우시나 표층토가 하층토의 토양수분에 영향을 주는 항우이전에 포장용수량(Field capacity)에 달했기 때문에 급항하는 지표면의 물 분자가 열에너지에 의해 증발산되고 천층에 산재한 초, 수근에 의해 소비되는 때문인 것으로 사료된다. 4. 지하수위하의 토양수분은 포화에 달하여 거의 일정할 것으로 기대되었지만 실제로 많은 변화를 가져왔다. 이들 변화에는 부분적으로 토양사극이 지하수위하이라도 채워지지 않았거나(Capillary pressure가 작을 시) 혹은 관측기간 중에 토양의 융기와 수축에 기인된다고 생각된다. 5. 지하배수구가 지표배수구에 비해 지하수위항하가 빨랐음은 물론 사질로움 토양에서는 보다 높은 투수계수로 인해서 지하수위의 항하가 점토질 토양에서 보다 훨씬 빨랐음을 보여주고 있다.표시할 수 있다.된다.acid $0.41{\sim}0.65%$, 오미자는 malic acid $1.51{\sim}3.90%$, citric acid $2.40{\sim}3.92%$로 주요 유기산이 있다. 타닌은 물보다 에탄올 추출물이 다소 함량이 높았으며 특히 오갈피는 $3.35{\sim}3.85%$로 매우 높은 함량이었다.a 6 cases, etc. 2. The retroperitoneal space including kidneys were the most commonly involved site (43.5%), of which Wilms' tumor was the commonest. 3. About 2/3 of tumors developed under the age of 6 and the commonest lesion was Wilms' tumor and the next being neuroblastoma, teratoma, ovarian cyst and so forth. 4. In all tumors except ovarian tumor and choledochal cyst, male was more frequently affected. 5. In plain abdomen 75% of neuroblastoma crossed the mid line while in Wilms' tumor only 2 cases (14%) showed midline cross. Calcific density was 입력불가 in all 6 cases of teratoma (100%) as dense and discrete ossification, in 3 case of neuroblatoma (38%) characteristically in diffuse stippled appearance and in one case

• The Journal of the Korean bone and joint tumor society
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• v.4 no.2
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• pp.94-98
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• 1998

Clear cell sarcoma of the kidney (CCSK) is a rare malignant tumor of the renal origin in childhood, distinguished from Wilms tumor by its pathologic and clinical features. Bone metastasis is one of the characteristic clinical features. The common site of metastasis of the clear cell Sarcoma of the kidney is axial skeleton including skull, spines, ribs and femur. A cases of clear cell Sarcoma of the kidney presented to us, which solely metastasized to the hand bones without metastasizing to any other tissues including axial skeleton. We report this case with review of literatures.

• Radiation Oncology Journal
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• v.9 no.2
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• pp.325-331
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• 1991

Treatment results of 28 patients with Wilms' tumor who received radiation therapy at the Department of Therapeutic Radiology, Seoul National University Hospital from 1979 to 1988 were analyzed. The median follow-up period for the survivors was 40 months. The local control and overall survival rate at 3 years were 78.1$\%$ and 67.4$\%$, respectively. The local control was not affected by age. The local control rates for favorable histology (FH) and unfavorable histology (UH) were 83.3$\%$ and 62.5$\%$, respectively. In FH, the local control rates of stage II and III were not different ($83.3\%\;vs\;100.0\%$). In UH, the control rates of stage I/II and stage III were 83.3$\%$ and 0$\%$, respectively. Poor local control was correlated with involvement of lymph node ($50.0\%\;vs\;87.5\%$). Radiotherapy delayed 10 or more days after operation resulted in poorer local control than that without delay (p<0.05). Thus radiotherapy contributed to reduction of local recurrence in patients with high risk factor without increased severe complication. It is suggested that bulky unresectable mass might need a more intensified treatment.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.21
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• pp.9217-9223
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• 2014

Background: Today, leukemia is one of the biggest problems worldwide. The Wilms' tumor gene (WT1) and the vascular endothelial growth factor (VEGF) gene are highly expressed in patients with various cancers. This study concerned the relationship between expression of WT1 and VEGF in patients with acute leukemia. Materials and Methods: We evaluated expression of WT1 mRNA and VEGF mRNA using real-time quantitative RT-PCR in the peripheral blood (PB) of 8 newly diagnosed AML and 4 newly diagnosed ALL patients, serially monitored for 2 months. A further 12 normal PB samples served as controls. Results: In the patient group, in comparison with the normal ranges, WT1 and VEGF gene expression was increased, the average values for the expression of these two genes being $0.2852{\pm}0.11$ and $0.2029{\pm}0.018$, respectively. While was no significant relevance between the two genes pre-treatment, a positive link between the two genes in 75% of patients with AML was noted during the procedure of chemotherapy, whereas in 75% of patients with ALL an antiparallel association was observed. Conclusions: Leukemia is associated with production of WT1, which may affect the expression of VEGF.

• The Korean Journal of Cytopathology
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• v.7 no.1
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• pp.69-74
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• 1996

Malignant rhabdold tumor is a distinct renal tumor in the pediatric age group. It was originally described as a rhabdomyosarcomatold variant of Wilms' tumor. However, subsequent studies fatted to confirm myogenous differentiation, so it is now considered to be a distinct and unique type of highly malignant tumor, histogenetically unrelated. Although extrarenal forms of this tumor are rare, several examples have been described in other sites, especially the liver, prostate, paravertebral area, urinary bladder and soft tissue. We experienced a case of malignant rhabdiod tumor located in the intraabdominal cavity in a 10 month-old boy. Smear of peritoneal fluid showed round, polygonal and irregular shaped cells with large nuclei, ample cytoplasm containing light pink to purple cytoplasmic inclusions, and one or a few prominent nucleoli. Immunocytochemistry revealed positivity to cytokeratin, epithelial membrane antigen and vimentin, and negativity to desmin and neuron-specific enolase. These distinct cytologic appearance and immunophenotypes were most consistent with a diagnosis of extrarenal malignant rhabdoid tumor. The cytoplasmic inclusions were correlated with eosinophilic inclusions seen in histologic section and electron microscopy confirmed this interpretation, showing filamentous aggregations in the cytoplasms of the tumor cells.

• Advances in pediatric surgery
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• v.5 no.1
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• pp.45-52
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• 1999

Pediatric solid tumors have many histologic similarity. These tumors contained small round cell types, and cause frequent diagnostic problems in pediatric pathology. An important advance in the differentiation of these small round cell tumors has been the identification of consistent chromosomal translocations associated with several types of tumors. Eighteen patients with soft tissue sarcoma were available for review. Seventeen cell lines were also included in this study. The RNA from the specimens were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). PAX3-FKHR fusion was present in four of five alveolar rhabdomyosarcoma and PAX7-FKHR fusion was detected in one of five alveolar rhabdomyosarcoma. None of the specimens expressed more than one chimeric transcript. EWS-FLI1 or EWS-ERG fusions were detected in all seven Ewings' sarcoma. No specimens showed EWS-WT1 fusion. These results corresponded well to the histopathologic diagnosis. There were no differences in the histologic appearances of tumors with the more frequent PAX3-FKHR or EWS-FLI1 fusions compared with those containing the variant PAX7-FKHR or EWS-ERG fusions. RT-PCR assay for chimeric transcript is a useful tool for rapid and objective diagnosis of pediatric solid tumors. Through these tools, we can approach genetically to the differential diagnosis of undifferentiated small round tumors.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.1-4
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• 2016

Congenital aniridia is a rare ocular malformation that presents with severe hypoplasia of the iris and various ocular manifestations. Most cases of congenital aniridia are known to be related to mutations in the paired box gene-6 (PAX6 ), which is an essential gene in eye development. Herein, we report a familial case of autosomal dominant congenital aniridia with four affected members in 3 consecutive generations and describe the detailed ophthalmologic findings for one of these members. As expected, mutational analysis revealed a nonsense mutation (p.Ser122*) in the PAX6 gene. Thus, our findings reiterate the importance of PAX6 mutations in congenital aniridia.