BACKGROUND The research findings suggest that the prognosis of children with Wilms tumor(WT)is affected by various factors.Some scholars have indicated that loss of heterozygosity(LOH)on chromosome 16q is associated w...BACKGROUND The research findings suggest that the prognosis of children with Wilms tumor(WT)is affected by various factors.Some scholars have indicated that loss of heterozygosity(LOH)on chromosome 16q is associated with a poor prognosis in patients with WT.AIM To further elucidate this relationship,we conducted a meta-analysis.METHODS This meta-analysis was registered in INPLASY(INPLASY2023100060).We systematically searched databases including Embase,PubMed,Web of Science,Cochrane,and Google Scholar up to May 31,2020,for randomized trials reporting any intrapartum fetal surveillance approach.The meta-analysis was performed within a frequentist framework,and the quality and network inconsistency of trials were assessed.Odds ratios and 95%CIs were calculated to report the relationship between event-free survival and 16q LOH in patients with WT.RESULTS Eleven cohort studies were included in this meta-analysis to estimate the relationship between event-free survival and 16q LOH in patients with WT(I^(2)=25%,P<0.001).As expected,16q LOH can serve as an effective predictor of eventfree survival in patients with WT(risk ratio=1.95,95%CI:1.52–2.49,P<0.001).CONCLUSION In pediatric patients with WT,there exists a partial correlation between 16q LOH and an unfavorable treatment prognosis.Clinical detection of 16q chromosome LOH warrants increased attention to the patient’s prognosis.展开更多
The relationship between genetic alterations at chromosomal band 11p13 and the WAGR(Wilms'tumor,aniridia,genitourinary anomalies,and mental retardation)syndrome is not clearly understood.To aid our understanding o...The relationship between genetic alterations at chromosomal band 11p13 and the WAGR(Wilms'tumor,aniridia,genitourinary anomalies,and mental retardation)syndrome is not clearly understood.To aid our understanding of this relationship,we have constructed a physical map of this region of the genome using pulsed field gel electrophoresis.Fifteen newly identified 11p13-specific probes and four previously reported probes were used to subdivide 11p13 into five intervals defined by overlapping constitutional deletions from several WAGR patients.This new repertoire of DNA probes was used to construct a physical map of this region using the infrequently cutting restriction enzymes MIuI and NotI.This map spans approximately 13 Mb and encompasses deletion and translocation breakpoints associated with genitourinary abnormalities,aniridia,and Wilms'tumor.The map also makes it possible to localize the genes for Wilms'tumor(WT)and aniridia(AN2)to a small number of specific NotI restriction fragments.展开更多
Background: Wilms’ tumor (WT), the most common malignant neoplasm of the urinary tract of children [1], accounts for 5.9% of childhood cancers and affects one in every 10,000 children worldwide before the age of 15 y...Background: Wilms’ tumor (WT), the most common malignant neoplasm of the urinary tract of children [1], accounts for 5.9% of childhood cancers and affects one in every 10,000 children worldwide before the age of 15 years. The care of children with Wilm’s tumor in sub-Saharan Africa is compromised due to resource deficiencies that range from inadequate healthcare budgets to paucity of appropriately trained personnel. Childhood Wilms tumor is surging as an important paediatric problem in developing and sub-Saharan Africa countries. The objective of the study is to establish an understanding on the treatment challenges and outcomes of Wilm’s tumor in South West Ethiopia. Results: Forty-three Wilm’s tumor patients who were admitted from January 2017 to December 2021 were included in the study. The most frequent presentation was painless abdominal swelling in 40 (93%) patients. Fourteen patients (32.6%) were hypertensive at the time of diagnosis and the other 13 (30.2%) were normal. In abdominal examination, 31 (72.1%) patients had abdominal mass not crossing the midline and 12 (27.9%) had mass crossing the midline. After multimodal treatment, 37.5% had improvement, 11.6% came back with relapse. Most patients (41.7%) abandoned treatment and 9.3% of the cohort died in the course of treatment. Conclusion: The outcomes in the treatment of Wilms Tumor have been found to be poor in this review. The main reason for poor outcome has been not receiving adequate chemotherapy after surgery. Doses of chemotherapy received after surgery significantly affected treatment outcomes (p = 0.026).展开更多
Objective:The incidence of Wilms’tumor(WT)among adult individuals accounts for less than 1%of kidney cancer cases,with a prognosis usually less favorable when compared to younger individuals and an overall survival r...Objective:The incidence of Wilms’tumor(WT)among adult individuals accounts for less than 1%of kidney cancer cases,with a prognosis usually less favorable when compared to younger individuals and an overall survival rate of 70%for the adult patients versus 90%for the pediatric cases.The diagnosis and treatment of WT are complex in the preoperative setting;neoadjuvant chemotherapy(NAC)or robotic surgery has rarely been described.This study aimed to review the literature of robotic surgery in WT and report the first adult WT management using both NAC and robotic strategy.Methods:We reported a case of WT managed in a multidisciplinary setting.Furthermore,according to Preferred Reporting Items for Systematic reviews and Meta-Analyses recommendations,a systematic review of the literature until August 2020 of WT treated with a robotic approach was carried out.Results:A 33-year-old female had a diagnosis of WT.She was scheduled to NAC,and according to the clinical and radiological response to a robotic radical nephrectomy with aortic lymph nodes dissection,she was managed with no intraoperative rupture,a favorable surgical outcome,and a follow-up of 25 months,which did not show any recurrence.The systematic review identified a total number of 230 cases of minimally invasive surgery reported in the literature for WT.Of these,approximately 15 patients were carried out using robotic surgery in adolescents while none in adults.Moreover,NAC has not been administered before minimally invasive surgery in adults up until now.Conclusion:WT is a rare condition in adults with only a few cases treated with either NAC or minimally invasive approach so far.The advantage of NAC followed by the robotic approach could lead to favorable outcomes in this complex scenario.Notwithstanding,additional cases of adult WT need to be identified and investigated to improve the oncological outcome.展开更多
Severely immunocompromised NOD.Cg-PrkdcIl2rg(NOG)mice are among the ideal animal recipients for generation of human cancer models.Transplantation of human solid tumors having abundant tumor-i nfiltrating lymphocytes(T...Severely immunocompromised NOD.Cg-PrkdcIl2rg(NOG)mice are among the ideal animal recipients for generation of human cancer models.Transplantation of human solid tumors having abundant tumor-i nfiltrating lymphocytes(TILs)can induce xenogeneic graft-versus-host disease(xGvHD)following engraftment and expansion of the TILs inside the animal body.Wilms’tumor(WT)has not been recognized as a lymphocyte-predominant tumor.However,3 consecutive generations of NOG mice bearing WT patient-derived xenografts(PDX)xenotransplanted from a single donor showed different degrees of inflammatory symptoms after transplantation before any therapeutic intervention.In the initial generation,dermatitis,auto-amputation of digits,weight loss,lymphadenopathy,hepatitis,and interstitial pneumonitis were observed.Despite antibiotic treatment,no response was noticed,and thus the animals were prematurely euthanized(day 47 posttransplantation).Laboratory and histopathologic evaluations revealed lymphoid infiltrates positively immunostained with anti-human CD3 and CD8 antibodies in the xenografts and primary tumor,whereas no microbial infection or lymphoproliferative disorder was found.Mice of the next generation that lived longer(91 days)developed sclerotic skin changes and more severe pneumonitis.Cutaneous symptoms were milder in the last generation.The xenografts of the last 2 generations also contained TILs,and lacked lymphoproliferative transformation.The systemic immunoinflammatory syndrome in the absence of microbial infection and posttransplant lymphoproliferative disorder was suggestive of xGvHD.While there are few reports of xGvHD in severely immunodeficient mice xenotransplanted from lymphodominant tumor xenografts,this report for the first time documented serial xGvHD in consecutive passages of WT PDX-bearing models and discussed potential solutions to prevent such an undesired complication.展开更多
Aim:The purpose of the present study was to perform a comprehensive analysis of WT1 gene expression in high-risk pediatric acute lymphoblastic leukemia(ALL).Methods:We performed a meta-analysis of WT1 gene expression ...Aim:The purpose of the present study was to perform a comprehensive analysis of WT1 gene expression in high-risk pediatric acute lymphoblastic leukemia(ALL).Methods:We performed a meta-analysis of WT1 gene expression for normal hematopoietic cells vs.primary leukemia cells from 801 pediatric ALL samples deposited in the Oncomine database combined with an in-depth gene expression analysis using our in-house database of gene expression profiles of primary leukemia cells from 1416 pediatric ALL cases.We also examined the expression of WT1 in primary leukemic cells from 299 T-lineage ALL patients in the Oncomine database and 189 T-lineage ALL patients in the archived datasets GSE13159,GSE13351,and GSE13159.Results:Our data provide unprecedented evidence that primary leukemia cells from patients with MLL gene rearrangements(MLL-R)express highest levels of WT1 expression within the high-risk subsets of pediatric B-lineage ALL.Notably,MLL-R^(+)patients exhibited>6-fold higher expression levels of the WT1 gene compared to the other B-lineage ALL subtypes combined(P<0.0001).Our findings in 97 MLL-R^(+)infant B-lineage ALL cases uniquely demonstrated that WT1 is expressed at 1.5-4.2-fold higher levels in MLL-R^(+)infant leukemia cells than in normal hematopoietic cells and revealed that WT1 expression level was substantially higher in steroid-resistant infant leukemia cells when compared to non-leukemic healthy bone marrow cells.Furthermore,our study demonstrates for the first time that the WT1-regulated EWSR1,TP53,U2AF2,and WTAP genes(i.e.,WT1 interactome)were differentially upregulated in MLL-R^(+)leukemia cells illustrating that the MLL-regulatory pathway is aberrantly upregulated in MLL-R^(+)pediatric B-lineage ALL.These novel insights provide a compelling rationale for targeting WT1 in second line treatment of MLL-R^(+)pediatric B-lineage ALL,including MLL-R^(+)infant ALL.Furthermore,our study is the first to demonstrate that leukemia cells from 370 Ph-like patients had significantly higher WT1 expression when compared to normal hematopoietic cells.Finally,our findings demonstrate for the first time that chemotherapy-resistant primarily leukemic cells from relapsed B-lineage ALL patients exhibit higher expression levels of WT1 than primary leukemia cells from newly diagnosed B-lineage ALL patients(P=0.001).Conclusion:Our findings indicate that the WT1 gene product may serve as a target for immunotherapy in high risk/poor prognosis subsets of newly diagnosed as well as relapsed pediatric B-lineage ALL.Our findings also significantly expand the current knowledge of WT1 expression in T-lineage ALL and provide new evidence that WT1 gene and its interactome are expressed in T-lineage ALL cells at significantly higher levels than in normal hematopoietic cells.This previously unknown differential expression profile uniquely indicates that the protein product of WT1 would be an attractive molecular target for treatment of T-lineage ALL as well.展开更多
We collected a medical record of“Wilms’tumor”in the Department of Urology at the Third Affiliated Hospital of Inner Mongolia Medical University,and discussed the diagnosis,auxiliary examination and treatment of the...We collected a medical record of“Wilms’tumor”in the Department of Urology at the Third Affiliated Hospital of Inner Mongolia Medical University,and discussed the diagnosis,auxiliary examination and treatment of the disease.We hope to expand clinical thinking,improve our diagnosis and treatment of the disease through data analysis.展开更多
Extrarenal Wilms’ Tumors (ERWTs) are rare. There have been only 25 cases of ERWT arising from the female genital system reported in the literature. In this paper, we report a 60-year-old woman with a complaint of vag...Extrarenal Wilms’ Tumors (ERWTs) are rare. There have been only 25 cases of ERWT arising from the female genital system reported in the literature. In this paper, we report a 60-year-old woman with a complaint of vaginal bleeding and a polypoid mass in the uterine cavity by sonography that was demonstrated as ERWT by pathology after resection. The pathological characteristics, histological origination,diagnosis, therapy and prognosis of ERWT in female reproductive system are discussed in this paper in the purpose of improving the diagnosis and therapy of this rare tumor.展开更多
We studied 22 Wilms’tumors of children immunohistochemically.We’ve found that the positive rate of p53 in slices was 31.8％ (7),of nm23 was 50％ (11),and of p16 was 86.4％ (19).It suggested that mutation rate of p53...We studied 22 Wilms’tumors of children immunohistochemically.We’ve found that the positive rate of p53 in slices was 31.8％ (7),of nm23 was 50％ (11),and of p16 was 86.4％ (19).It suggested that mutation rate of p53 was high in tumors,expression of nm23 in favorite histology(FH)was higher than that in unfavorite histology(UFH) group,and p16 showed very high positive rate in tumors.All of the three showed no relation with sex,age,or pathological type.So each one may be useful in clinic to evaluate pathogenesis and prognosis.展开更多
BACKGROUND Wilms tumor is the most common renal malignancy in childhood. It occurs primarily between the ages of 2 and 5 years. The usual manifestations are abdominal mass, hypertension, and hematuria. The case presen...BACKGROUND Wilms tumor is the most common renal malignancy in childhood. It occurs primarily between the ages of 2 and 5 years. The usual manifestations are abdominal mass, hypertension, and hematuria. The case presented here had an unusual presentation, with dilated cardiomyopathy and hypertension secondary to the Wilms tumor. CASE SUMMARY A 3-year-old boy presented with a 5-d history of irritability, poor appetite, and respiratory distress. His presenting clinical symptoms were dyspnea, tachycardia, hypertension, and a palpable abdominal mass at the left upper quadrant. His troponin T and pro-B-type natriuretic peptide levels were elevated. Echocardiography demonstrated a dilated hypokinetic left ventricle with an ejection fraction of 29%, and a suspected left renal mass. Computed tomography scan revealed a left renal mass and multiple lung nodules. The definitive diagnosis of Wilms tumor was confirmed histologically. The patient was administered neoadjuvant chemotherapy and underwent radical nephrectomy. After surgery, radiotherapy was administered, and the adjuvant chemotherapy was continued. The blood pressure and left ventricular function normalized after the treatments. CONCLUSION Abdominal mass, dilated cardiomyopathy and hypertension can indicate Wilms tumor in pediatric patients. Chemotherapy and tumor removal achieve successful treatment.展开更多
Osteopathia striata with cranial sclerosis(OSCS, OMIM#300373) is an X-linked dominant sclerosing bone dysplasia that shows a distinct phenotype in females and males. In 2009, Zandra Jenkins et al found that germline m...Osteopathia striata with cranial sclerosis(OSCS, OMIM#300373) is an X-linked dominant sclerosing bone dysplasia that shows a distinct phenotype in females and males. In 2009, Zandra Jenkins et al found that germline mutations in the FAM123 B /WTX /AMER1 gene, mapped to chromosome Xq11.2, cause both the familial and sporadic forms of OSCS. Intriguingly, the WTX gene was already known as a putative tumor suppressor gene, since in 2007 Rivera et al had reported inactivating WTX mutations in Wilms' tumor(WT), the most frequent renal tumor of childhood. Here we review the heterogeneous clinical presentation of OSCS patients and the involvement of WTX anomalies in OSCS and in WT.展开更多
基金Supported by Yunnan Provincial Department of Science and Technology Provincial Basic Research Program(Kunming Medical Joint Special Project,No.2019FE001(-276)Kunming Health Science and Technology Talents Training Project and"Ten Hundred Thousands"Project Training Plan,No.2020-SW(Backup)-121.
文摘BACKGROUND The research findings suggest that the prognosis of children with Wilms tumor(WT)is affected by various factors.Some scholars have indicated that loss of heterozygosity(LOH)on chromosome 16q is associated with a poor prognosis in patients with WT.AIM To further elucidate this relationship,we conducted a meta-analysis.METHODS This meta-analysis was registered in INPLASY(INPLASY2023100060).We systematically searched databases including Embase,PubMed,Web of Science,Cochrane,and Google Scholar up to May 31,2020,for randomized trials reporting any intrapartum fetal surveillance approach.The meta-analysis was performed within a frequentist framework,and the quality and network inconsistency of trials were assessed.Odds ratios and 95%CIs were calculated to report the relationship between event-free survival and 16q LOH in patients with WT.RESULTS Eleven cohort studies were included in this meta-analysis to estimate the relationship between event-free survival and 16q LOH in patients with WT(I^(2)=25%,P<0.001).As expected,16q LOH can serve as an effective predictor of eventfree survival in patients with WT(risk ratio=1.95,95%CI:1.52–2.49,P<0.001).CONCLUSION In pediatric patients with WT,there exists a partial correlation between 16q LOH and an unfavorable treatment prognosis.Clinical detection of 16q chromosome LOH warrants increased attention to the patient’s prognosis.
文摘The relationship between genetic alterations at chromosomal band 11p13 and the WAGR(Wilms'tumor,aniridia,genitourinary anomalies,and mental retardation)syndrome is not clearly understood.To aid our understanding of this relationship,we have constructed a physical map of this region of the genome using pulsed field gel electrophoresis.Fifteen newly identified 11p13-specific probes and four previously reported probes were used to subdivide 11p13 into five intervals defined by overlapping constitutional deletions from several WAGR patients.This new repertoire of DNA probes was used to construct a physical map of this region using the infrequently cutting restriction enzymes MIuI and NotI.This map spans approximately 13 Mb and encompasses deletion and translocation breakpoints associated with genitourinary abnormalities,aniridia,and Wilms'tumor.The map also makes it possible to localize the genes for Wilms'tumor(WT)and aniridia(AN2)to a small number of specific NotI restriction fragments.
文摘Background: Wilms’ tumor (WT), the most common malignant neoplasm of the urinary tract of children [1], accounts for 5.9% of childhood cancers and affects one in every 10,000 children worldwide before the age of 15 years. The care of children with Wilm’s tumor in sub-Saharan Africa is compromised due to resource deficiencies that range from inadequate healthcare budgets to paucity of appropriately trained personnel. Childhood Wilms tumor is surging as an important paediatric problem in developing and sub-Saharan Africa countries. The objective of the study is to establish an understanding on the treatment challenges and outcomes of Wilm’s tumor in South West Ethiopia. Results: Forty-three Wilm’s tumor patients who were admitted from January 2017 to December 2021 were included in the study. The most frequent presentation was painless abdominal swelling in 40 (93%) patients. Fourteen patients (32.6%) were hypertensive at the time of diagnosis and the other 13 (30.2%) were normal. In abdominal examination, 31 (72.1%) patients had abdominal mass not crossing the midline and 12 (27.9%) had mass crossing the midline. After multimodal treatment, 37.5% had improvement, 11.6% came back with relapse. Most patients (41.7%) abandoned treatment and 9.3% of the cohort died in the course of treatment. Conclusion: The outcomes in the treatment of Wilms Tumor have been found to be poor in this review. The main reason for poor outcome has been not receiving adequate chemotherapy after surgery. Doses of chemotherapy received after surgery significantly affected treatment outcomes (p = 0.026).
文摘Objective:The incidence of Wilms’tumor(WT)among adult individuals accounts for less than 1%of kidney cancer cases,with a prognosis usually less favorable when compared to younger individuals and an overall survival rate of 70%for the adult patients versus 90%for the pediatric cases.The diagnosis and treatment of WT are complex in the preoperative setting;neoadjuvant chemotherapy(NAC)or robotic surgery has rarely been described.This study aimed to review the literature of robotic surgery in WT and report the first adult WT management using both NAC and robotic strategy.Methods:We reported a case of WT managed in a multidisciplinary setting.Furthermore,according to Preferred Reporting Items for Systematic reviews and Meta-Analyses recommendations,a systematic review of the literature until August 2020 of WT treated with a robotic approach was carried out.Results:A 33-year-old female had a diagnosis of WT.She was scheduled to NAC,and according to the clinical and radiological response to a robotic radical nephrectomy with aortic lymph nodes dissection,she was managed with no intraoperative rupture,a favorable surgical outcome,and a follow-up of 25 months,which did not show any recurrence.The systematic review identified a total number of 230 cases of minimally invasive surgery reported in the literature for WT.Of these,approximately 15 patients were carried out using robotic surgery in adolescents while none in adults.Moreover,NAC has not been administered before minimally invasive surgery in adults up until now.Conclusion:WT is a rare condition in adults with only a few cases treated with either NAC or minimally invasive approach so far.The advantage of NAC followed by the robotic approach could lead to favorable outcomes in this complex scenario.Notwithstanding,additional cases of adult WT need to be identified and investigated to improve the oncological outcome.
基金supported by the grant received from Tehran University of Medical Sciences(TUMS-38292)。
文摘Severely immunocompromised NOD.Cg-PrkdcIl2rg(NOG)mice are among the ideal animal recipients for generation of human cancer models.Transplantation of human solid tumors having abundant tumor-i nfiltrating lymphocytes(TILs)can induce xenogeneic graft-versus-host disease(xGvHD)following engraftment and expansion of the TILs inside the animal body.Wilms’tumor(WT)has not been recognized as a lymphocyte-predominant tumor.However,3 consecutive generations of NOG mice bearing WT patient-derived xenografts(PDX)xenotransplanted from a single donor showed different degrees of inflammatory symptoms after transplantation before any therapeutic intervention.In the initial generation,dermatitis,auto-amputation of digits,weight loss,lymphadenopathy,hepatitis,and interstitial pneumonitis were observed.Despite antibiotic treatment,no response was noticed,and thus the animals were prematurely euthanized(day 47 posttransplantation).Laboratory and histopathologic evaluations revealed lymphoid infiltrates positively immunostained with anti-human CD3 and CD8 antibodies in the xenografts and primary tumor,whereas no microbial infection or lymphoproliferative disorder was found.Mice of the next generation that lived longer(91 days)developed sclerotic skin changes and more severe pneumonitis.Cutaneous symptoms were milder in the last generation.The xenografts of the last 2 generations also contained TILs,and lacked lymphoproliferative transformation.The systemic immunoinflammatory syndrome in the absence of microbial infection and posttransplant lymphoproliferative disorder was suggestive of xGvHD.While there are few reports of xGvHD in severely immunodeficient mice xenotransplanted from lymphodominant tumor xenografts,this report for the first time documented serial xGvHD in consecutive passages of WT PDX-bearing models and discussed potential solutions to prevent such an undesired complication.
基金This work was supported by departmental funds of the Ares Pharmaceuticals Biotherapy Program.No external funding sources or sponsored research grants were used.
文摘Aim:The purpose of the present study was to perform a comprehensive analysis of WT1 gene expression in high-risk pediatric acute lymphoblastic leukemia(ALL).Methods:We performed a meta-analysis of WT1 gene expression for normal hematopoietic cells vs.primary leukemia cells from 801 pediatric ALL samples deposited in the Oncomine database combined with an in-depth gene expression analysis using our in-house database of gene expression profiles of primary leukemia cells from 1416 pediatric ALL cases.We also examined the expression of WT1 in primary leukemic cells from 299 T-lineage ALL patients in the Oncomine database and 189 T-lineage ALL patients in the archived datasets GSE13159,GSE13351,and GSE13159.Results:Our data provide unprecedented evidence that primary leukemia cells from patients with MLL gene rearrangements(MLL-R)express highest levels of WT1 expression within the high-risk subsets of pediatric B-lineage ALL.Notably,MLL-R^(+)patients exhibited>6-fold higher expression levels of the WT1 gene compared to the other B-lineage ALL subtypes combined(P<0.0001).Our findings in 97 MLL-R^(+)infant B-lineage ALL cases uniquely demonstrated that WT1 is expressed at 1.5-4.2-fold higher levels in MLL-R^(+)infant leukemia cells than in normal hematopoietic cells and revealed that WT1 expression level was substantially higher in steroid-resistant infant leukemia cells when compared to non-leukemic healthy bone marrow cells.Furthermore,our study demonstrates for the first time that the WT1-regulated EWSR1,TP53,U2AF2,and WTAP genes(i.e.,WT1 interactome)were differentially upregulated in MLL-R^(+)leukemia cells illustrating that the MLL-regulatory pathway is aberrantly upregulated in MLL-R^(+)pediatric B-lineage ALL.These novel insights provide a compelling rationale for targeting WT1 in second line treatment of MLL-R^(+)pediatric B-lineage ALL,including MLL-R^(+)infant ALL.Furthermore,our study is the first to demonstrate that leukemia cells from 370 Ph-like patients had significantly higher WT1 expression when compared to normal hematopoietic cells.Finally,our findings demonstrate for the first time that chemotherapy-resistant primarily leukemic cells from relapsed B-lineage ALL patients exhibit higher expression levels of WT1 than primary leukemia cells from newly diagnosed B-lineage ALL patients(P=0.001).Conclusion:Our findings indicate that the WT1 gene product may serve as a target for immunotherapy in high risk/poor prognosis subsets of newly diagnosed as well as relapsed pediatric B-lineage ALL.Our findings also significantly expand the current knowledge of WT1 expression in T-lineage ALL and provide new evidence that WT1 gene and its interactome are expressed in T-lineage ALL cells at significantly higher levels than in normal hematopoietic cells.This previously unknown differential expression profile uniquely indicates that the protein product of WT1 would be an attractive molecular target for treatment of T-lineage ALL as well.
文摘We collected a medical record of“Wilms’tumor”in the Department of Urology at the Third Affiliated Hospital of Inner Mongolia Medical University,and discussed the diagnosis,auxiliary examination and treatment of the disease.We hope to expand clinical thinking,improve our diagnosis and treatment of the disease through data analysis.
基金supported by the Science and Technology Program from Science and Technology Department of Shandong Province of China(2012G 0021820)
文摘Extrarenal Wilms’ Tumors (ERWTs) are rare. There have been only 25 cases of ERWT arising from the female genital system reported in the literature. In this paper, we report a 60-year-old woman with a complaint of vaginal bleeding and a polypoid mass in the uterine cavity by sonography that was demonstrated as ERWT by pathology after resection. The pathological characteristics, histological origination,diagnosis, therapy and prognosis of ERWT in female reproductive system are discussed in this paper in the purpose of improving the diagnosis and therapy of this rare tumor.
文摘We studied 22 Wilms’tumors of children immunohistochemically.We’ve found that the positive rate of p53 in slices was 31.8％ (7),of nm23 was 50％ (11),and of p16 was 86.4％ (19).It suggested that mutation rate of p53 was high in tumors,expression of nm23 in favorite histology(FH)was higher than that in unfavorite histology(UFH) group,and p16 showed very high positive rate in tumors.All of the three showed no relation with sex,age,or pathological type.So each one may be useful in clinic to evaluate pathogenesis and prognosis.
文摘BACKGROUND Wilms tumor is the most common renal malignancy in childhood. It occurs primarily between the ages of 2 and 5 years. The usual manifestations are abdominal mass, hypertension, and hematuria. The case presented here had an unusual presentation, with dilated cardiomyopathy and hypertension secondary to the Wilms tumor. CASE SUMMARY A 3-year-old boy presented with a 5-d history of irritability, poor appetite, and respiratory distress. His presenting clinical symptoms were dyspnea, tachycardia, hypertension, and a palpable abdominal mass at the left upper quadrant. His troponin T and pro-B-type natriuretic peptide levels were elevated. Echocardiography demonstrated a dilated hypokinetic left ventricle with an ejection fraction of 29%, and a suspected left renal mass. Computed tomography scan revealed a left renal mass and multiple lung nodules. The definitive diagnosis of Wilms tumor was confirmed histologically. The patient was administered neoadjuvant chemotherapy and underwent radical nephrectomy. After surgery, radiotherapy was administered, and the adjuvant chemotherapy was continued. The blood pressure and left ventricular function normalized after the treatments. CONCLUSION Abdominal mass, dilated cardiomyopathy and hypertension can indicate Wilms tumor in pediatric patients. Chemotherapy and tumor removal achieve successful treatment.
基金Supported by Associazione Bianca Garavaglia,21052 BustoArsizio,Varese,ItalyItalian Association for Cancer Research(AIRC)Fondazione Pierfranco e Luisa Mariani,20129 Milano,Italy
文摘Osteopathia striata with cranial sclerosis(OSCS, OMIM#300373) is an X-linked dominant sclerosing bone dysplasia that shows a distinct phenotype in females and males. In 2009, Zandra Jenkins et al found that germline mutations in the FAM123 B /WTX /AMER1 gene, mapped to chromosome Xq11.2, cause both the familial and sporadic forms of OSCS. Intriguingly, the WTX gene was already known as a putative tumor suppressor gene, since in 2007 Rivera et al had reported inactivating WTX mutations in Wilms' tumor(WT), the most frequent renal tumor of childhood. Here we review the heterogeneous clinical presentation of OSCS patients and the involvement of WTX anomalies in OSCS and in WT.
