Application value of energy spectrum CT multi-parameters in pathological classification of nephroblastoma / 实用放射学杂志

Objective To explore the application value of multi-parameters of energy spectrum CT in the pathological classification of nephroblastoma.Methods A total of 30 children with nephroblastoma who were underwent energy spectrum CT examination were enrolled in this study,retrospectively.The iodine(I)value and CT value of tumor and abdominal aorta were collected in three phases,respectively.The normalized iodine concentration(NIC),1 value enhancement ratio,CT value enhancement ratio and I value/CT value enhancement ratio were calculated and statistically analyzed.Results Statistical analysis showed that in the comparison of NIC and multi-parameters,there were statistical differences between the three types of nephroblastoma(P＜0.05).In the comparison of the slopes of energy spectrum curves,the slopes from large to small were respectively germ nephroblastoma,mixed nephroblastoma and mesenchymal nephroblastoma,and the differences were statistically significant(P＜0.05).Through receiver operating characteristic(ROC)curve analysis,it was found that the NIC and I value/CT value enhancement ratio and slope of energy spectrum curve in the identification of high-risk type and medium-risk type of nephroblastoma,the area under the curve(AUC)was 0.91-0.96,and the sensitivity was 75％-95％,and the specificity was above 90％.Conclusion Multi-parameters of energy spectrum CT can be used as the basis for the pathological classification of nephroblastoma.NIC and I value/CT value enhancement ratio and slope of energy spectrum curve can be used as differential indicators of high and medium-risk pathological types of nephroblastoma.

Effects of WTAPP1 on the proliferation,invasion,migration and Wnt/β-catenin signaling pathway of nephroblastoma cells / 肿瘤

Objective:To investigate the effects of long non-coding RNA Wilms tumor 1 associated protein pseudogene 1(WTAPP1)on the proliferation,invasion,migration and Wnt/β-catenin signaling pathway of nephroblastoma cells. Methods:Real-time fluorescence quantitative PCR was used to detect the relative expression level of WTAPP1 in 48 cases of nephroblastoma tumor tissues and their matched adjacent tissues,human nephroblastoma cells(SK-NEP-1)and normal renal epithelial cells(PCS-400-01 0,PCS-400-011 and PCS-400-01 2).The clinicopathological characteristics and prognosis of nephroblastoma patients with high or low WTAPP1 expression of were analyzed and compared.SK-NEP-1 cells were infected with lentivirus carrying the full-length WTAPP]gene(WTAPP1 overexpression)or shRNA targeting WTAPP1(shWTAPP1).Then,colony formation assay and CCK-8 assay were used to assess the proliferation of SK-NEP-1 cells,wound healing assay and Transwell assay were used to evaluate the migration and invasion activity of SK-NEP-1 cells respectively,and Western blotting was used to examine the relative expression levels of Wnt3a,β-catenin,C-myc and Survivin proteins. Results:The relative expression level of WTAPP1 in cancer tissues was higher than that in adjacent tissues(P＜0.05).The relative expression level of WTAPP1 in SK-NEP-1 cells was higher than that in normal renal epithelial cells(PCS-400-01 0,PCS-400-01 1 and PCS-400-01 2;P＜0.05).WTAPP1 expression was associated with the clinical stages of nephroblastoma(P＜0.05)but not with the age,gender,tumor diameter or lymph node metastasis of nephroblastoma patients(all P＞0.05).The 5-year survival rate of WTAPP1-high patients was significantly lower than that of WTAPP1-low patients(P＜0.05).The proliferation,migration and invasion activities of SK-NEP-1 cells were significantly increased after WTAPP1 overexpression(all P＜0.05)while decreased after WTAPP1 silencing(all P＜0.05).The relative expression levels of Wnt3a,β-catenin,C-myc and Survivin proteins were significantly upregulated after WTAPP1 overexpression(all P＜0.05)while decreased after WTAPP1 silencing(all P＜0.05). Conclusion:WTAPP1 is correlated with the clinical stages and prognosis of nephroblastoma patients and may promote the proliferation,invasion and migration of tumor cells by activating Wnt/β-catenin signaling pathway.

Correlation between 25-hydroxyvitamin D and nephroblastoma in children and its value in assessing disease prognosis / 中国当代儿科杂志

OBJECTIVES@#To study the correlation between 25-hydroxyvitamin D [25-(OH)D] and nephroblastoma in children and its value in assessing the prognosis of the disease.@*METHODS@#A total of 50 children with nephroblastoma who were admitted from January 2018 to December 2022 were included as the nephroblastoma group, and according to the postoperative pathological type, they were divided into a good prognosis group with 38 children and a poor prognosis group with 12 children. A total of 50 healthy children who underwent physical examination during the same period of time served as the healthy control group. The above groups were compared in terms of serum creatinine and 25-(OH)D level. A Spearman correlation analysis was used to investigate the correlation between serum 25-(OH)D level and therapeutic effect reaction. A multivariate logistic regression analysis was used to identify the risk factors affecting the prognosis of nephroblastoma in children.@*RESULTS@#The nephroblastoma group had significantly lower levels of serum creatinine and 25-(OH)D than the healthy control group (P<0.05). Compared with the good prognosis group, the poor prognosis group had a significantly larger tumor diameter, a significantly higher proportion of children with stage III-IV tumors, a significantly higher rate of tumor metastasis, and significantly lower serum levels of creatinine and 25-(OH)D (P<0.05). The Spearman correlation analysis showed that serum 25-(OH)D level was negatively correlated with therapeutic effect reaction (rs=-0.685, P<0.001). The multivariate logistic regression analysis showed that tumor diameter ≥10 cm, stage III-IV tumors, presence of tumor metastasis, and 25-(OH)D <19 ng/mL were closely associated with the poor prognosis of nephroblastoma in children (P<0.05). Serum 25-(OH)D level had an area under the curve of 0.805 (95%CI: 0.706-0.903, P<0.001) in evaluating the prognosis of nephroblastoma in children, with a Youden index of 0.512, a sensitivity of 0.938, and a specificity of 0.575 at the optimal cut-off value of 1.764 ng/mL.@*CONCLUSIONS@#There is a significant correlation between 25-(OH)D level and the prognosis of nephroblastoma in children, and 25-(OH)D can be used for prognosis prediction.

Differentiating pediatric cystic nephroma from common renal multicystic lesions: A case report

Pediatric cystic nephroma is a rare, clinically benign, renal tumor. Pediatric renal cystic lesions are complex. Imaging findings and tumor appearance are often nonspecific, and careful pathological examination is necessary. We discuss diagnosis of pediatric cystic nephroma and how to differentiate it from multicystic dysplastic kidney and cystic partially differentiated nephroblastoma.

Hallazgo necrópsico de nefroblastoma asociado a síndrome metabólico experimental / Necropsic finding of nephroblastoma associated to experimental metabolic syndrome

Introducción: El síndrome metabólico presenta entre sus manifestaciones la obesidad, la cual se encuentra relacionada con el desarrollo de cáncer. Sin embargo, no habíamos encontrado en cuatro años ningún caso de neoplasias malignas en animales con síndrome metabólico. Objetivo: Describir el desarrollo de un tumor maligno a nivel renal en modelo experimental de síndrome metabólico. Métodos: El biomodelo experimental se logró por la aplicación de una solución de sacarosa al 35 por ciento, durante 20 semanas a 50 ratas machos Wistar destetados. El diagnóstico de nefroblastoma se realizó mediante necropsia con observación morfológica de la lesión renal. Resultados: Confirmado el síndrome metabólico se detectó en un caso, una masa palpable en abdomen. En la inspección macroscópica se observó un tumor en el polo inferior del riñón derecho, color pardo grisáceo, con hemorragia y cambios quísticos. Histológicamente se observaron alteraciones propias de un nefroblastoma mixto con componentes del blastema, mesenquimal y epitelial. Conclusiones: Se describe por vez primera, en estudio anatomopatológico, la presencia de un caso de nefroblastoma en rata con síndrome metabólico experimental(AU)

Introduction: One of the manifestations of metabolic syndrome is obesity, which is in turn related to the development of cancer. However, in four years we had not found any case of malignant neoplasms in animals with metabolic syndrome. Objective: Describe the development of a malignant renal tumor in an experimental metabolic syndrome model. Methods: The experimental biomodel was made applying a 35 percent saccharose solution to 50 male weaned Wistar rats for 20 weeks. The diagnosis of nephroblastoma was achieved by necropsy with morphological observation of the renal lesion. Results: Upon metabolic syndrome confirmation, a palpable mass was detected in the abdomen of one of the cases. Macroscopic observation revealed a grayish brown tumor in the lower pole of the right kidney with hemorrhaging and cystic changes. Histological examination found alterations typical of mixed nephroblastoma with blastema, mesenchymal and epithelial components. Conclusions: This is the first time a description is provided in an anatomopathological study of a case of nephroblastoma in a rat with experimental metabolic syndrome(AU)

MicroRNA-200c-3p inhibits proliferation of nephroblastoma cells by targeting CCNE2 / 南方医科大学学报

OBJECTIVE@#To predict and verify the target gene of miR-200c-3p and evaluate the inhibitory effect of miR-200c-3p on the proliferation of nephroblastoma cells.@*METHODS@#The putative target genes of miR-200c-3p were predicted by bioinformatics approach. Nephroblastoma cell models with miR-200c-3p overexpression or knockdown were established in SK-NEP-1 and G401 cells with corresponding control groups. The expressions of CCNE2 in SK-NEP-1 and G401 cells in different groups were detected by RT-PCR and Western blotting. A luciferase reporter assay was used to determine the targeting relationship between miR-200c-3p and CCNE2. The effects of miR-200c-3p overexpression or knockdown on cell proliferation was detected by cell counting kit-8 (CCK-8) assay and soft agarose assay.@*RESULTS@#CCNE2 was one of the target genes of miR-200c-3p as predicted by bioinformatics methods. Transfection of the two nephroblastoma cell lines with miR-200c-3p mimic resulted in significantly lowered CCNE2 mRNA and protein expressions ( < 0.05). The results of dual-luciferase assay confirmed that miR-200c-3p bound to the 3'UTR of CCNE2. CCK-8 assay and soft agarose assay demonstrated that overexpression of miR-200c-3p significantly inhibited the proliferation of the nephroblastoma cells ( < 0.01), and knocking down miR-200c-3p in the cells produced the opposite effects.@*CONCLUSIONS@#miR-200c-3p overexpression inhibits the proliferation of nephroblastoma cells by down-regulating its target gene CCNE2.

Factores asociados a la sobrevida en pacientes con tumor de Wilms / Factors associated with survival in patient with Wilms tumor

Introducción. El tumor de Wilms es el segundo tumor abdominal más frecuente en la edad pediátrica y responde por más del 90 % de los tumores renales en pediatría. A pesar de que la sobrevida descrita es mayor del 90 %, en nuestro medio encontramos que solo alcanza al 70 %, por lo que deseamos evaluar cuáles son los factores asociados con dichos resultados desfavorables, con el fin de implementar medidas para mejorar la sobrevida de nuestros pacientes. Métodos. Se realizó un estudio observacional, transversal, en dos centros de alto nivel de atención, que incluyó una muestra de 84 pacientes menores de 15 años, con diagnóstico de tumor de Wilms. Resultados. Los factores que se asociaron significativamente con un aumento en la probabilidad de morir fueron: no completar el protocolo de quimioterapia, (OR 34; IC95% 3,7-312; p 0,000) y presentar recidiva tumoral (OR 35,7; IC95% 6,9-184; p 0,000). Otros factores que aumentaron esta probabilidad sin alcanzar a ser significativos, pero mostrando una evidente tendencia fueron: presentación bilateral (OR 4,1; IC95% 0,6-5,5; p 0,147), complicaciones quirúrgicas (OR 3,2; IC95% 0,7-14,6; p 0,136), compromiso de ganglios linfáticos en tomografía (OR 2,4; IC95% 0,7-8,4; p 0,139) y las metástasis a distancia (OR 2,5; IC95% 0,7-9; p 0,143). Discusión. La sobrevida de nuestros niños con tumor de Wilms es menor que la reportada en la literatura mundial, siendo la falla en terminar la quimioterapia, la recidiva y la necesidad de cirugía bilateral, los factores asociados con este desenlace

Introduction. Wilms tumor is the second most frequent abdominal tumor in pediatric age, and it accounts for more than 90% of kidney tumors in pediatrics. Although the described survival is greater than 90%, in our set-ting we find that it only reaches 70%. Our objective was to evaluate the factors associated with these unfavorable results, in order to implement measures to improve the survival of our patients.Methods. An observational, cross-sectional study was conducted in two tertiary medical centers, which included a sample of 84 patients under 15 years of age with a diagnosis of Wilms tumor.Results. The factors that were significantly associated with an increase in the probability of dying were not com-pleting the chemotherapy protocol (OR 34; 95%CI 3.7-312; p 0.000) and presenting tumor recurrence (OR 35.7; 95%CI 6.9-184; p 0.000). Other factors that increased this probability without being significant, but showing an evident trend were: bilateral presentation (OR 4.1; 95%CI 0.6-5.5; p 0.147), surgical complications (OR 3.2; 95%CI 0.7-14.6; p 0.136), lymph node involvement in tomography (OR 2.4; 95%CI 0.7-8.4; p 0.139) and distant metastases (OR 2.5; 95%CI 0.7-9; p 0.143).Discussion. The survival of the children with Wilms tumor in our study was lower than that reported in the world literature, with failure to complete chemotherapy, recurrence and the need for bilateral surgery being the factors associated with this outcome

Tumor de Wilms no adulto: neoplasia rara e um desafio diagnóstico e terapêutico / Wilms tumor in adult: a rare neoplasia and a diagnostic and therapeutic challenge

O tumor de Wilms é o tumor renal maligno mais comum na criança. Sua apresentação em adultos é rara. Atualmente, há relato de aproximadamente 250 casos de tumor de Wilms em adultos. Relata-se neste trabalho, um novo caso em paciente do sexo masculino, 18 anos, com histórico de dor lombar direita e hematúria. A ressonância nuclear magnética de pelve demonstrou volumosa lesão expansiva com epicentro no terço médio do rim direito. Tomografia computadorizada de tórax com múltiplos nódulos esparsos pelo parênquima pulmonar, sugestivos de implantes secundários. Foi realizada nefrectomia radical direita e o estudo anatomo-patológico mais imuno-histoquimica confirmaram o diagnóstico de nefroblastoma (Tumor de Wilms) em estágio IV. Embora se tenha conseguido uma boa resposta com os esquemas de tratamento quimioterápicos atuais, estudos mostram que o prognóstico do tumor de Wilms em adultos é inferior quando comparado ao pediátrico. O qual pode estar relacionado ao fato de se tratar de uma doença rara com diagnóstico tardio.

Wilms tumor is the most common malignant renal tumor in children. His presentation in adults is rare. Currently, there are approximately 250 cases of Wilms tumor in adults. This paper reports a new case in a male patient, 18 years old, with a history of right lower back pain and hematuria. Pelvic Nuclear magnetic resonance demonstrated a massive expansive lesion with epicenter in the middle third of the right kidney. Computed tomography of the chest with multiple nodules scattered by the pulmonary parenchyma, suggestive of secondary implants. Right radical nephrectomy was performed and the anatomopathological and immunohistochemical study confirmed the diagnosis of nephroblastoma (Wilms' tumor) in stage IV. Although a good response has been achieved with current chemotherapy regimens, studies have shown that Wilms tumor prognosis in adults is lower when compared to pediatric. This may be related to the fact that it is a rare disease with a late diagnosis.

Mechanism of tumor suppressor gene DKK2 inhibiting proliferation of nephroblastoma cells in children / 实用肿瘤学杂志

Objective The aims of this study were to investigate the expression of DKK2,a WNT signaling pathway regula-tor,in nephroblastoma cells and tissues of children,the effect on the proliferation of nephroblastoma SK-NEP-1 cells,and to explore its mechanism. Methods The relative expression of DKK2 in nephroblastoma cells and tissues was analyzed by qRT-PCR and im-munoblotting assays. Overexpressing DKK2 SK-NEP-1 cells were set as the experimental group( DKK2 group);the blank control plasmid group was set as a control group( Vector group),transfected with pcDNA3. 1 ( +) -Flag-DKK2 plasmid( Experimental group)and pcDNA3. 1( +) -Flag-Vector plasmid(Control group). The over-expression of DKK2 was confirmed in SK-NEP-1 cells by RT-PCR and immunoblotting. CCK-8 and cell cloning assays were used to determine the effect of DKK2 on cell prolifera-tion;flow cell cycle and apoptosis assays were used to confirm the effect on cell proliferation in overexpressed DKK2 cells. The xen-graft formation assay in nude mice was to verify the effect of DKK2 on proliferation in overexpressed DKK2 cells;the mechanism of DKK2 in inhibitory proliferation was analyzed by qRT-PCR,Western blotting and immunohistochemistry. Results Compared with normal renal epithelial tissues,DKK2 mRNA was down-regulated in children with nephroblastoma,and the difference was statistically significant(P<0. 001). Compared with the control group,transfected DKK2 cell viability was significantly inhibited after treatment for 24,48 and 72 h( P<0. 05),cell clone formation in the experimental group was significantly inhibited(31. 11% ± 2. 14% ) ( P<0. 05),the cell cycle in the experimental group was significantly arrested at the G1 phase(P<0. 001),and the apoptosis rate in the experimental group was significantly increased(P<0. 001). Compared with the control group,the tumor weight and volume in nude mice were significantly low in the experimental mice which were injected DKK2 overexpression cells(P<0. 001). Active-β-cate-nin and downstream genes were significantly inhibited in over-expressed DKK2 SK-NEP-1 cells. Conclusion DKK2 is down-regulated in human cutaneous nephroblastoma and participates in the mechanism of nephroblastoma by antagonizing Wnt/β-catenin signaling pathway.

Nephroblastoma in a black-tufted marmoset (Callithrix penicillata) / Nefroblastoma em um sagui-de-tufos-pretos (Callithrix penicillata)

A renal nephroblastoma is described in a free-living black-tufted marmoset (Callithrix penicillata) in Central Brazil. The monkey was found dead and subjected to necropsy. Gross anatomic changes consisted of a ruptured left kidney, which was almost completely effaced by a white to yellow, partially encapsulated friable mass. The left ureter was distended due to obstruction by a red, spherical, 2mm in diameter friable mass. The urinary bladder was also distended. Histologically the renal and ureteral masses consisted of a triphasic embryonal neoplasm composed of embryonic epithelium forming glomeruli and tubules, polygonal blastemal cells, and a mesenchymal stroma. The embryonic epithelium exhibited rare nuclear immunoreactivity for WT-1, whereas blastemal cells exhibited robust cytoplasmic and rare nuclear immunoreactivity for WT-1; blastemal cells were also immunoreactive for vimentin. No immunoreactivity was detected for pan-cytokeratin (AE1/AE3), actin, and desmin. Morphological and immunohistochemical features of the present neoplasm are consistent with those described for renal nephroblastoma.(AU)

Descreve-se um caso de nefroblastoma maligno em um sagui de vida livre no Brasil Central. O macaco foi encontrado morto e encaminhado para necropsia. Na macroscopia, o rim esquerdo apresentava-se rompido e o parênquima estava substituído por um tecido neoplásico friável, parcialmente encapsulado e de superfície natural branca e de corte amarela. O ureter esquerdo apresentava-se distendido devido à obstrução por uma massa friável, vermelha, esférica, de 2mm de diâmetro. Histologicamente, as massas renal e ureteral consistiam de uma neoplasia embrionária composta por três populaçõies de células neoplásicas, composta por epitélio embrionário formando glomérulos e túbulos, células blastemais poligonais e um estroma mesenquimal. O epitélio embrionário exibiu imunorreactividade nuclear rara para WT-1, enquanto que as células blastemais exibiram imunorreactividade nuclear citoplasmática e rara para WT-1; As células blastemais também foram imunorreativas à vimentina. Nenhuma imunorreatividade foi detectada para pan-citoqueratina (AE1/AE3), actina e desmina. As características morfológicas e imuno-histoquímicas da presente neoplasia são consistentes com as descritas para o nefroblastoma renal.(AU)