ULTRASTRUCTURAL CHARACTERISTICS OF NEPHROBLASTOMAS AND THEIR RELATION TO DNA PLOID

Fifty-one cases of nephroblastoma were pathologically studied by light and electron microscopy as well as microspectrophotometry. Under light and electron microscope, the tumor appeared to be carcinosarcoma structurally, consisting of epithelial, sarcomatous and undlfferentiated tumor cells. Their ultrastructural feature was similar to that of renal tubular epithelial cells and tibroblast. It could differentially be diagnosed from renal metoblastic tumors by electron microscopy when the tumor was mainly composed of sarcomatous cells. Microspectrophotometer measurement showed an Increased mean nuclear DNA content (DI>1. 04), belonging to aneuplold tumor, which together with polyploid cells, serving as one of the Important characteristics of malignant tumors. In addition, the poorer the tumor differentiation the higher was the DNA content of the tumor cells, suggesting that the measurement of nuclear DNA of tumor cells might also serve as one of signs to acertain the degree of differentiation and malignant of nephroblastomas with more preciseneas than general morphological examinations.

Factors of Nephroblastomas’ Relapse in Children at the Academic Hospital Gabriel Toure, Bamako, Mali

Introduction:?The factors of nephroblastomas’ relapse are a set of elements playing a role in the reappearance of cancer cells in the same place of the kidney or in other regions of the body after a 5-year remission.?Objectives:?To determine the frequency and the factors of nephroblastomas’ relapse in the pediatric oncology unit and pediatric surgery of the academic hospital Gabriel Touré. Materials and methods:?This was a retrospective study carried out from January 1, 2005 to December 31, 2019 in all children treated for nephroblastoma relapses in the pediatric oncology unit and in the pediatric surgery service.?Results:?In 15 years, we have managed 182 cases of nephroblastoma, of which 128 cases were declared in complete remission after 5 years and 12 cases presented a recurrence. The relapses factors were: capsular break, intraoperative tumor rupture, presence of an associated malformation, surgery without neoadjuvant chemotherapy and tumor surgical stage (p??0.05). Patients’ age at diagnosis, sex, number of neoadjuvant and adjuvant chemotherapy sessions and histological type were not decisive (p?> 0.05).?Conclusion:?Relapses are more and more frequent in cases of nephroblastoma. The correct use of the protocol would avoid these recurrences.

Nephroblastoma in Adults about a Clinical Case

Nephroblastoma is the most common malignant renal tumor in children and is related to an abnormal proliferation of cells resembling those of the embryonic kidney (metanephroma), hence the terminology;embryonal tumor. These are tumors that remain and remain unstudied in Mali because they are common in adults in our context. Its annual incidence is estimated at approximately 1/10,000 births. Nephroblastoma is a rare or even exceptional tumor in adults. The clinical manifestation was a large swelling of the right hypochondrium;abdominal pain for a year;unquantified fever, hypertension, initial hematuria associated with burning during urination and anemia. The main clinical manifestation remained fever and abdominal pain. This renal tumor posed a diagnostic problem which was previously labeled as a mesenteric tumor in our general surgery department. The diagnosis was made by imaging: CT and magnetic resonance imaging. The treatment is multidisciplinary and combines chemotherapy, surgery with or without radiotherapy. The prognosis is poor due to late diagnosis and less effectiveness of chemotherapy compared to the child. Survival did not exceed a year and a half because the renal tumor in our patient was surgically overcome. We report a case of nephroblastoma in an 86-year-old patient with unfavorable histology (hematogenous metastases), operated on in the general surgery department and whose postoperative course was simple and who was referred to oncology for treatment.

Surgical Treatment of Nephroblastoma in a 4-Year Male Child: A Case Report and Literature Review

Herein we report a patient with nephroblastoma which was successfully removed at the Sunyani Teaching Hospital CJ Oppong theatre in Ghana in sub-Saharan Africa by extended below umbilical incision. Our patient had a family history of Wilms tumour predisposing him to the disease. His main symptoms were haematuria and abdominal mass which was noticed later. Examination and investigation were suggestive of a late-stage unilateral Nephroblastoma resulting in the need for nephrectomy. To reduce the need for such radical surgeries among children at an early stage, there is the need for early screening of children for Wilms tumours especially, those with family predisposition as in our case study. The case report presented here constitutes a rare case of nephroblastoma in the literature.

Robotic approach with neoadjuvant chemotherapy in adult Wilms’ tumor: A feasibility study report and a systematic review of the literature

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.

Cyr61/CTGF/Nov family proteins in gastric carcinogenesis

Gastric cancer(GC)is the second leading cause of cancer-related death.The poor survival rate may reflect the relatively aggressive tumor biology of GC.Recently,the importance of the tumor microenvironment in carcinogenesis has emerged.In the tumor microenvironment,tumor cells and the surrounding stromal cells aberrantly secrete matricellular proteins capable of modulating carcinogenesis and regulating metastasis.The Cyr61/CTGF/Nov(CCN)proteins are a family of matricellular proteins with variable roles in many physiological and pathological processes.The evidence suggests that CCN family proteins contribute to GC carcinogenic processes.Here,we briefly review recent research on the effects of CCN family proteins in GC carcinogenesis and the development of new targeted agents in this field.

Study on the influence of curcumin on chemosensitivity of nephroblastoma cells

Objective:To study the influence of curcumin on chemosensitivity of nephroblastoma cells.Methods:Human nephroblastoma cells line SK-NEP-1 was transplanted to the nude mice subcutaneously to establish the implantation tumor model of human nephroblastoma cells.A total of 30 tumor-bearing mice were divided into three groups of ten randomly.The routine chemotherapy group was given vincristine(0.05 mg/mL·0.2 mL/d) and actinomycin D(15 ng/mL·0.2 mL/d) combined chemotherapy regime.The curcumin chemotherapy group was given the same combined chemotherapy regimens and curcumin(30 mg/kg/d) by intraperitoneal injection.The control group was given normal saline(NS) of the same volume by intraperitoneal injection.Continuous administration would be kept for 4 weeks and 3 days a week.The volumetric changes of every group were recorded.The serum of every group in different time was collected and the VEGF content was detected by ELISA.All mice were cercrificed and the tumor tissues were stripped and weighed after 4 weeks’ treatment.The tumor inhibition rate was calculated.The cell proliferation activity and apoptosis rate were detected by MTT and flow cytometry method.All data were statistically analyzed by SPSS 19.0.Results:The tumor volume,serum VEGF content,tumor inhibition rate,cell proliferation activity and apoptosis rate of routine chemotherapy group and curcumin chemotherapy group had significant differences comparing with the control group(P<0.05) after 4-week’s treatment.The cancer growth of curcumin chemotherapy group was obviously decreased and even tended to shrink comparing with routine chemotherapy group(χ2=15.732,P=0.007).The cell proliferation activity was significantly reduced and the apoptosis rate was significantly higher,(χ~2=9.427,P=0.012)which showing the effect of chemotherapy was enhanced.Conclusions:The chemosensitivity of nephroblastoma cells could be improved by curcumin,then the effect of preoperative adjuvant chemotherapy scheme would be enhanced,the growth of nephroblastoma cells would be inhibited and the surgical risk of nephroblastoma would be reduced.

Prevalence and Prognosis of Relapse of Nephroblastoma at the Pediatric Oncology Unit of Bamako

The nephroblastoma is the third pediatric cancer in Mali, this study aimed to describe the prevalence and prognosis of nephroblastoma relapses. Methods: It was a descriptive retrospective study over a 10-year period from January 2005 to March 2015. We collected children aged 0 to 15 years followed for relapse of nephroblastoma in the pediatric oncology unit of university hospital center (UHC) Gabriel Toure. Results: The frequency of relapse of nephroblastoma was 7.4% (19 cases) whose mean age was 42 months with a sex ratio of 1.3. The relapse occurred before the end of the postoperative course in 16% of cases (3 patients). It was local recurrence in 52% of cases (10 patients), pulmonary 16% (3 patients), and hepatic 11% (2 patients). According to the SIOP classification, 47% of patients were diagnosed in stage III (9 patients) and 21% (4 patients) in stage IV. The tumor was high risk in 37%. Palliative chemotherapy was performed in 63% of the patients (12 patients) and the remaining seven were put on a high risk diet. The overall survival at 5 years was 21% or 4 patients. Conclusion: Our results showed all the difficulties in the management of nephroblastoma relapses in our context.

Early Management of Nephroblastoma: A Single-Center Study in a Sub-Saharan African Country

Introduction: Nephroblastoma is the most common renal cancer in pediatrics. The aim of this study was to describe the epidemiological, clinical, histological characteristics and the immediate outcome of patients with nephroblastoma. Methodology: This was a cross-sectional study of 18 cases of unilateral nephroblastoma, carried out in the pediatric oncology unit (UOP) of the CHU Gabriel Touré from January 2015 to December 2016. Results: The mean age of the patients was 33 months old. The sex ratio was 0.63. The average consultation time was 3 months. A case of malformation syndrome (aniridia and mental retardation) had been observed. The main reason for consultation was abdominal mass (100%) associated with deterioration of general condition (44%), pain (44%) and fever (17%). Stage I accounted for 61% and stage II 39%. The postoperative histological classification made it possible to find the high-risk blastematous type (33%), the regressive type of intermediate risk (11%) and the stromal type of intermediate risk (45%). Toxicities (haematological and digestive) were minimal. The 3-year overall survival was 90%. Conclusion: This study is characterized by the predominance of stages I and II and the fairly large percentage of histology at intermediate risk. These results are above all the result of multidisciplinary collaboration.

Nephroblastoma: Radiological and Pathological Diagnosis of a Case with Liver Metastases

Nephroblastoma is one of the most common causes of abdominal-pelvic mass in children. It still raises a diagnostic problem because of the wide variety of causes of abdominal-pelvic mass but also because of the delays in consultation in sub-Saharan black Africa and consequently the discovery of the mass at a very late and sometimes metastatic stage. Yet nephroblastoma is a very chemo-sensitive malignancy requiring diagnosis at an early stage;a procedure in which medical imaging is essential. We report the case of a large abdominal-pelvic mass in a three-year-old girl in whom the abdominal-pelvic CT allowed to diagnose nephroblastoma with liver metastases. Our objective is to demonstrate the contribution of computed tomography in the diagnosis of large abdominal-pelvic mass of the child and discuss other causes of abdominal-pelvic mass of the child.

Correlation of TGF-β and IGF-1 signaling pathway expression in nephroblastoma tissue with cell proliferation activity and invasion activity

Objective: To study the correlation of TGF-β and IGF-1 signaling pathway expression in nephroblastoma tissue with cell proliferation activity and invasion activity. Methods:Nephroblastoma tumor tissue and adjacent tissue surgically removed in Renmin Hospital of Wuhan University between May 2014 and March 2017 were selected, the expression levels of TGF-β and IGF-1 signal pathway molecules were determined after protein extraction, and the mRNA expression levels of cell proliferation genes and invasion genes were determined after RNA extraction. Results: TGFBR1, IGF1R, p-ERK, Smad2/3 and p-AKT protein expression levels in nephroblastoma tumor tissue were significantly higher than those in adjacent tissue;DNp73, Survivin, Ki-67, TUBB3, MMP2 and N-cadherin mRNA expression levels in nephroblastoma tumor tissue were significantly higher than those in adjacent tissue and positively correlated with TGFBR1, IGF1R, p-ERK, Smad2/3 and p-AKT protein expression levels while Bmi, TAp73, TIMP1, TIMP2 and E-cadherin mRNA expression levels were significantly lower than those in adjacent tissue and negatively correlated with TGFBR1, IGF1R, p-ERK, Smad2/3 and p-AKT protein expression levels. Conclusion: The high expression of TGF-β and IGF-1 signaling pathways in nephroblastoma tissue can promote the proliferation and invasion of tumor cells.

Identification of a constitutional mutation in the WT1 gene in Taiwan Residents patients with Wilms tumor

The overall frequency of WT1 gene alterations in Wilms tumor is still unclear in Taiwan. Here we conducted molecular genetic analysis of the WT1 gene in Taiwan Residents patients with Wilms tumor. Polymerase chain reaction and direct sequencing were performed on DNA samples from blood and paraffin-embedded tumor specimens. A constitutional mutation in the WT1 gene was found in one DNA sample from peripheral blood lymphocytes. The remaining DNA samples from peripheral blood lymphocytes and paraffin-embedded tumor specimens were tested negative for both constitutional mutations and somatic mutations. Thus, mutations at other Wilms tumor loci may play an important role in Wilms tumor development.

Correlation of nephroblastoma overexpressed gene (NOV) mRNA levels with cell growth- and migration-related gene expression in osteosarcoma tissue

Objective:To study the correlation of nephroblastoma overexpressed gene (NOV) mRNA levels with cell growth- and migration-related gene expression in osteosarcoma tissue. Methods: A total of 78 patients with osteosarcoma undergoing surgical treatment in our hospital between May 2012 and May 2016 were selected, and the osteosarcoma tissue and paracancerous tissue were collected. Fluorescence quantitative PCR method was used to detect the mRNA expression of NOV as well as pro-proliferation/anti-proliferation genes and invasion genes, and the Pearson test was used to further assess the correlation of NOV mRNA expression with the osteosarcoma cell growth and migration activity.Results: NOV mRNA expression in osteosarcoma tissue was significantly lower than that in paracancerous tissue, FoxM1, STIM1 and Orai1 mRNA expression were significantly higher than those in paracancerous tissue, anti-proliferation genes RanBP9, Tap73, Caspase-3, PTEN and P53 mRNA expression were significantly lower than those in paracancerous tissue, and invasion genes EFEMP1, Notch1, Id1 and Src mRNA expression were significantly higher than those in paracancerous tissue. Pearson test showed that the NOV mRNA expression in osteosarcoma tissue was negatively correlated with pro-proliferation genes FoxM1, STIM1 and Orai1 mRNA expression, positively correlated with anti-proliferation genes RanBP9, Tap73, Caspase-3, PTEN and P53 mRNA expression, and negatively correlated with invasion genes EFEMP1, Notch1, Id1 and Src mRNA expression.Conclusion: NOV expression is relatively low in osteosarcoma tissue, and NOV mRNA expression is negatively correlated with tumor cell proliferation and invasion activity.

Experimental study about the regulating effect of Par-4 gene overexpression on the nephroblastoma sensitivity to cisplatin

Objective: To study the regulating effect of Par-4 gene overexpression on the nephroblastoma sensitivity to cisplatin. Methods: Nephroblastoma SK-NEP-1 cells were cultured and divided into four groups, control group were treated with RMPI-1640 without serum or drugs, cisplatin group were treated with serum-free RMPI-1640 containing 5 μg/mL cisplatin, Par-4 group were transfected by Par-4 overexpression plasmids with serum-free RMPI-1640, and cisplatin+ Par-4 group were transfected by Par-4 overexpression plasmid with serum-free RMPI-1640 containing 5 μg/mL cisplatin. The cell proliferation activity as well the expression of apoptosis genes, migration genes and invasion genes was measured. Results: 8 h, 16 h and 24 h after different conditions of treatment, the cell proliferation activity of cisplatin group, Par-4 group and cisplatin + Par-4 group were significantly lower than that of control group, and the cell proliferation activity of cisplatin + Par-4 group was significantly lower than that of cisplatin group and Par-4 group;24 h after different conditions of treatment, Bim, PDCD4, WT1, RGS4, Axin, KAI1, E-cadherin, PPARγ and PTEN mRNA expression in cisplatin group, Par-4 group and cisplatin + Par-4 group were greatly higher than those in control group whereas GDNF, GFRα1, TUBB3, NME1 and FGF1 mRNA expression were greatly lower than those in control group;Bim, PDCD4, WT1, RGS4, Axin, KAI1, E-cadherin, PPARγ and PTEN mRNA expression in cisplatin + Par-4 group were greatly higher than those in cisplatin group and Par-4 group whereas GDNF, GFRα1, TUBB3, NME1 and FGF1 mRNA expression were greatly significantly lower than those in cisplatin group and Par-4 group. Conclusion:Par-4 gene overexpression can increase the nephroblastoma sensitivity to cisplatin, reduce cell proliferation activity, promote apoptosis and inhibit cell migration and invasion.

Developmental study on the immunocytochemical localization of NOV protein-containing neurons in the rat brain

The localization and development of nephroblastoma overexpressed gene (nov) protein-immunoreactive neurons in the brain of E8-P300 rats have been studied using immunocytochemistry and image analysis. Results are as follows: No NOV protein-immunoreactive cells were detected in the rat brain during prenatal development. A few of positive cells were detected at the early postnatal stage. However, the number and the immunoreactivity of these cells increased gradually at later stages. NOV-immunoreactive cells were widely distributed in the rat brain during P30-P60. The number of immunoreactive cells and their intensity also peaked within this stage. The number and staining intensity of NOV-positive cells decreased gradually with age. The positive cells were mainly located in cingulate cortex, striatum, hippocampus, hypothalamus, cerebellum and brain stem. The present results indicate that nov may play an important role in the development and differentiation of brain as well as maintaining the function of

Prevention of tumor emboli from the inferior vena cava by the Tempofilter II during resection of nephroblastoma with level III tumor thrombus

The optimal treatment for patients with nephroblastoma and inferior vena cava （IVC） minor thrombus is radical nephrectomy and minor thrombectomv, but the operation for patients with level III tumor thrombus is usually at high risk of puhnonary, embolism （PE）. We report one case of nephroblastoma with level III thrombus in our hospital in 2007, the vena cava tumor thrombectonly was safely performed under the protection of Tempofilter II inferior vena cava filter.

Analysis of the unexpected damage in children Wilm○s tumor

Objective: To explore the relationship between unexpected damage and clinical character, biological activities and molecular biology. Methods: We treated 76 cases of children wilms tumor from 1995 to 1998. Among them, 9 cases (11.8%) underwent unexpected damages. We studied and analyzed the clinical characters, clinical pathology, morphology and molecular biology in the 9 cases. Results: Wilms tumor in late stage, blastoma cell type and bad differentiation was likely to be damaged unexpectedly, and the outcome was poor. Conclusions: Mutation of gene is related to the biological activities. It may be a potential and important factor to cause unexpected damage.