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Wilms tumour is named after Max Wilms. It is an embryonal tumour derived from the metanephros. It is the commonest childhood renal tumour and the third commonest paediatric malignancy. Synchronous bilateral Wilms tumours (BWT) represent 4–7% of all Wilms tumours (WT) and present at a younger age than unilateral Wilms tumours. At least 10% of synchr...
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Wilms tumour is named after Max Wilms. It is an embryonal tumour derived from the metanephros. It is the commonest childhood renal tumour and the third commonest paediatric malignancy. Synchronous bilateral Wilms tumours (BWT) represent 4-7% of all Wilms tumours (WT) and present at a younger age than unilateral Wilms tumours. At least 10% of synchr...
Citations
... Wilms tumor (WT) is one of the most common pediatric malignant tumors in clinical practice, originating from the metanephros containing epithelial, blastocyst, and interstitial tissue (Millar et al. 2017). As one of the successes of pediatric oncology, a good cure rate for WT can be achieved with the use of relatively simple treatments (Spreafico and Bellani 2006). ...
miRNA has been a research hotspot in recent years and its scope of action is very wide, involving the regulation of cell proliferation, differentiation, apoptosis, and other biological behaviors. This study intends to explore the role of miRNA in the lipid metabolism and development of Wilms tumor (WT) by detecting and analyzing the differences in the expression profiles of miRNAs between the tumor and adjacent normal tissue. Gene detection was performed in tumor tissues and adjacent normal tissues of three cases of WT to screen differentially expressed miRNAs (DEMs). According to our previous research, FASN, which participates in the lipid metabolism pathway, may be a target of WT. The starBase database was used to predict FASN-targeted miRNAs. The above two groups of miRNAs were intersected to obtain FASN-targeted DEMs and then GO Ontology (GO) functional enrichment analysis of FASN-targeted DEMs was performed. Finally, the FASN-targeted DEMs were compared and further verified by qRT‒PCR. Through gene sequencing and differential analysis, 287 DEMs were obtained, including 132 upregulated and 155 downregulated miRNAs. The top ten DEMs were all downregulated. Fourteen miRNAs targeted by the lipid metabolism-related gene FASN were predicted by starBase. After intersection with the DEMs, three miRNAs were finally obtained, namely, miR-107, miR-27a-3p, and miR-335-5p. GO enrichment analysis was mainly concentrated in the Parkin-FBXW7-Cul1 ubiquitin ligase complex and response to prostaglandin E. Further experimental verification showed that miR-27a-3p was significantly correlated with WT (P = 0.0018). Imbalanced expression of miRNAs may be involved in the occurrence and development of WT through lipid metabolism. The expression of miR-27a-3p is related to the malignant degree of WT, and it may become the target of diagnosis, prognosis, and treatment of WT in the later stage.
... Wilms tumor (WT), the most common kidney carcinoma in children, is an embryonic carcinoma without a definitive cure (Millar et al., 2017). According to a recent study, Septin 2 (SEPT2) loss-of-function in G-401 cells (WT cells) reduced proliferation, migration, and invasion while inducing apoptosis emphasizing the role of SEPT2 on tumorigenesis in WT. ...
Mesenchymal stromal/stem cell-(MSC-) derived exosomes are gaining popularity for their involvement in tissue repair and repressing various tumors through extensive patterns. Nevertheless, the impact of extracellular vesicles produced by stem cells on tumor formation and progression is controversial and seems to depend on several factors. The utilization of MSCs' various capabilities in urogenital neoplasms is widely regarded as a potential future therapeutic as well. These genitourinary neoplasms include prostatic neoplasms, ovarian neoplasms, cervical neoplasms, endometrial neoplasms, bladder neoplasms, and renal cell neoplasms. The present study has concentrated on the most recent information on genitourinary neoplasms employing MSCs derived exosomes' many capabilities, such as delivering effective RNAs, extensive tissue compatibility, and specificity with tumor identification without inherent limitations of cell therapy. CITATION Salehpour A, Balmagambetova S, Mussin N, Kaliyev A and Rahmanifar F (2023), Mesenchymal stromal/stem cell-derived exosomes and genitourinary cancers: A mini review.
... Wilms tumor (WT), the most common kidney carcinoma in children, is an embryonic carcinoma without a definitive cure (Millar et al., 2017). According to a recent study, Septin 2 (SEPT2) loss-of-function in G-401 cells (WT cells) reduced proliferation, migration, and invasion while inducing apoptosis emphasizing the role of SEPT2 on tumorigenesis in WT. ...
Mesenchymal stromal/stem cell- (MSC-) derived exosomes are gaining popularity for their involvement in tissue repair and repressing various tumors through extensive patterns. Nevertheless, the impact of extracellular vesicles produced by stem cells on tumor formation and progression is controversial and seems to depend on several factors. The utilization of MSCs’ various capabilities in urogenital neoplasms is widely regarded as a potential future therapeutic as well. These genitourinary neoplasms include prostatic neoplasms, ovarian neoplasms, cervical neoplasms, endometrial neoplasms, bladder neoplasms, and renal cell neoplasms. The present study has concentrated on the most recent information on genitourinary neoplasms employing MSCs derived exosomes’ many capabilities, such as delivering effective RNAs, extensive tissue compatibility, and specificity with tumor identification without inherent limitations of cell therapy.
... In cases of synchronous or potentially metachronous bilateral WT, renal tissue can be preserved by NSS. Best conditions for successful NSS are low volume tumors in the kidney's periphery (67,68). Neoadjuvant chemotherapy to confine the tumor enabling surgical resectability is therefore part of all treatment protocols. ...
... Neoadjuvant chemotherapy to confine the tumor enabling surgical resectability is therefore part of all treatment protocols. In a selected group of patients with bilateral WT, bilateral nephrectomy and kidney transplantation may be considered as a treatment option to eliminate the risk of initial or metachronous WT development (68,69). This subgroup includes patients with Denys-Drash syndrome in whom prophylactic or secondary bilateral nephrectomy (in case of ESRD) is an acceptable procedure (64,70,71). ...
Significant progress has been made in the management of Wilms tumor (WT) in recent years, mostly as a result of collaborative efforts and the implementation of protocol-driven, multimodal therapy. This article offers a comprehensive overview of current multidisciplinary treatment strategies for WT, whilst also addressing recent technical innovations including nephron-sparing surgery (NSS) and minimally invasive approaches. In addition, surgical concepts for the treatment of metastatic disease, advances in tumor imaging technology and potentially prognostic biomarkers will be discussed. Current evidence suggests that, in experienced hands and selected cases, laparoscopic radical nephrectomy and laparoscopic-assisted partial nephrectomy for WT may offer the same outcome as the traditional open approach. While NSS is the standard procedure for bilateral WT, NSS has evolved as an alternative technique in patients with smaller unilateral WT and in cases with imminent renal failure. Metastatic disease of the lung or liver that is associated with WT is preferably treated with a three-drug chemotherapy and local radiation therapy. However, surgical sampling of lung nodules may be advisable in persistent nodules before whole lung irradiation is commenced. Several tumor markers such as loss of heterozygosity of chromosomes 1p/16q, 11p15 and gain of function at 1q are associated with an increased risk of recurrence or a decreased risk of overall survival in patients with WT. In summary, complete resection with tumor-free margins remains the primary surgical aim in WT, while NSS and minimally invasive approaches are only suitable in a subset of patients with smaller WT and low-risk disease. In the future, advances in tumor imaging technology may assist the surgeon in defining surgical resection margins and additional biomarkers may emerge as targets for development of new diagnostic tests and potential therapies.
... Wilms tumor (WT) is an embryonic tumor derived from metanephros, and the most prevalent kidney tumor in childhood [1]. WT tends to grow into the vena cava and even invades the atrium, which increases the difficulty of surgery and elicits surgical complications [2]. ...
The exact mechanism of miR-15a-5p shuttled by human umbilical cord-mesenchymal stem cell-derived exosomes (hUC-MSCs-Exo) in Wilms tumor (WT) was estimated. WT tissues were collected clinically. miR-15a-5p and septin 2 (SEPT2) expression levels were examined in tissues . hUC-MSCs-Exo were transfected with miR-15a-5p-related oligonucleotides and co-cultured with WT cells (G-401). In addition, SEPT2 loss-of-function was performed in G-401 cells. The biological functions of G-401 cells after treatments were evaluated. Moreover, tumor formation tests further assessed the role of exosomal miR-15a-5p in WT. The miR-15a-5p level was lower and the SEPT2 level was higher in WT. hUC-MSCs-Exo impaired the biological functions of G-401 cells. hUC-MSCs-Exo carried upregulated miR-15a-5p into G-401 cells, thereby lessening the tumorigenic properties of G-401 cells. Inhibition of SEPT2 suppressed the biological function of WT cells and upregulated SEPT2 reversed hUC-MSCs-Exo-mediated inhibition of G-401 cell growth. The tumorigenicity of G-401 cells in mice was impaired by hUC-MSCs-Exo overexpressing miR-15a-5p. The data prove that miR-15a-5p shuttled by hUC-MSCs-Exo negatively regulates SEPT2 expression, and disrupts WT cell growth in vivo and in vitro.
... WT is prevalent on a global scale, although there are significant differences in the associated morbidity and prognosis [5]. The treatment regimen for WT usually includes surgery, chemotherapy, and radiotherapy [6] and the long-term survival outcomes for pediatric WT patients have gradually improved over the last several decades. However, specific subgroups of patients, such as those with relapse and anaplastic histology, have poor event-free survival and are at risk of developing significant late effects in response to therapy [7,8]. ...
Wilms tumor (WT) is one of the most common pediatric solid tumors, affecting 1 in 10,000 children, worldwide. A subset of WT patients has poor prognosis, which is associated with a high risk of advanced and/or recurrent disease. Therefore, candidate markers are urgently needed for the diagnosis and effective treatment of WT. We evaluated three mRNA microarray datasets to identify the differences between normal kidney tissue and WT tissue. Gene expression profiling revealed 130 differentially expressed genes (DEGs). Enrichment analysis and gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analyses were performed for the DEGs. Subsequently, we established a protein-protein interaction (PPI) network to reveal the associations among the DEGs and selected 10 hub genes, all of which were downregulated in WT. The expression of COL4A3, COL4A4, KCNJ1, MME, and SLC12A1 in WT tissues was significantly lower than that in normal renal tissues. Survival analyses using the Kaplan-Meier method showed that patients with WT and low expression of COL4A3, COL4A4, and KCNJ1 exhibited remarkably poor overall survival. The correlations among COL4A3, COL4A4, and KCNJ1 in WT were analyzed using cBioPortal; COL4A3, COL4A4, and KCNJ1 were positively correlated with each other. Thus, these genes were considered clinically significant and might therefore play important roles in carcinogenesis and the development of WT.
... Other options include wedge resection and enucleation (8) (see Figure 2). The traditional approach to PN is through a trans-abdominal incision (3,22), although a retroperitoneal approach has been described (23). The kidney should be fully mobilized on the vascular and ureteric pedicle, and then walled off from the peritoneum using large abdominal swabs (3) or by surrounding the kidney with sterile plastic (22). ...
... The traditional approach to PN is through a trans-abdominal incision (3,22), although a retroperitoneal approach has been described (23). The kidney should be fully mobilized on the vascular and ureteric pedicle, and then walled off from the peritoneum using large abdominal swabs (3) or by surrounding the kidney with sterile plastic (22). Vessel loops should be placed around the vascular pedicle to facilitate control in case of bleeding, even if the surgeon does not intend to clamp the vessels. ...
... A bloodless field is essential to safe surgery, and this can be achieved by simply placing bulldog or other vascular clamps on the hilar vessels. Many surgeons avoid clamping off the vascular pedicle entirely however, opting instead to occlude the pedicle with a finger (22), or to digitally compress the renal parenchyma proximal to the tumor (3). The administration of intravenous Mannitol 5-7 minutes prior to vascular occlusion may prevent ischemic renal damage by decreasing intracellular edema and intrarenal resistance (3,24). ...
Overall survival (OS) for children with Wilms tumor (WT) currently stands at around 90%. This is markedly improved from the survival rates of around 30% reported in the middle of the last century. This improvement is due to the development of multimodal treatment for this disease, based on the evidence yielded through international collaboration on trials conducted by the Société Internationale d'Oncologie Pédiatrique (SIOP) and the Children's Oncology Group (COG). In this article, we review some of the current surgical controversies surrounding the management of WT.
... Wilms tumor [WT] is the commonest malignant renal neoplasm in children, and it is also considered as the third most frequently encountered malignancy in the pediatric age group [1]. The management of WT is a true story of success regarding the multimodal treatment in children's oncology. ...
Background
Wilms tumor is the commonest malignant renal neoplasm in children. Surgery plays a pivotal role in the management, and evidence-based guidelines for surgical resection have been established by the major international groups. Any deviation from the protocol is considered as a violation. The goal of this study was to evaluate outcomes of the patients with unilateral Wilms tumor treated at a developing country and to analyze surgical violations (SV) and their impact on the prognosis. A retrospective review was conducted for 37 patients who were presented to our hospitals and underwent nephrectomy for WT from January 2016 to December 2018. All participating centers adopt Children’s Oncology Group protocol. The SV were analyzed by logistic regression. Overall survival (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier method.
Results
There were 12 (32.4%), 11 (29.7%), 10 (27%), and 4 (10.8%) stages I, II, III, and IV, respectively. Their median age at time of diagnosis was 3.1 years. Upfront nephrectomy was performed for 30 cases. Six patients had tumor relapse (2 lungs and 4 local recurrences) at a median follow-up of 15.7 months. Out of the relapsed patients, two had unfavorable histology, and regarding their staging, four were stage III, one was stage II, and one was stage IV. Thirty-month OS and EFS were 84.3% and 81.1%, respectively. Twenty-seven SV occurred within 25 patients. Lack or inadequate lymph node sampling represented 74.07% (20/27), intraoperative tumor rupture and spillage accounted for 18.52% (5/27), and unwarranted preoperative biopsy happened in 7.41% (2/27). The SV were not correlated with mortality (p value = 0.381); however, they had a significant impact on the relapse (p value = 0.001). On further analysis; tumor rupture and spillage was a predictor for recurrence reaching a statistical significance (p value = 0.003), whereas the other violations were not.
Conclusions
Favorable outcomes could be achieved by compliance with evidence-based guidelines even in a resource-limited country like ours. Violations were correlated with relapse; however, only tumor rupture and spillage was of statistical significance in multivariate analysis. Failure of lymph node documentation was the main problem encountered, and it should be avoidable in future practice.
... It originates from renal embryonic cells. WT mainly affects children younger than 15 years, with the incidence of 1/10000 [1][2][3][4] . Relevant multi-center trials uncovered that in kidney neoplasm patients younger than 7 months old, about 66% cases are WT, and the remaining 34% non-WT cases include congenital mesoblastic nephroma (18%), malignant rhabdoid rumor (8%), clear cell sarcoma of kidney (2%), and tumors of unknown histological type (6%) 3,4 . ...
... WT mainly affects children younger than 15 years, with the incidence of 1/10000 [1][2][3][4] . Relevant multi-center trials uncovered that in kidney neoplasm patients younger than 7 months old, about 66% cases are WT, and the remaining 34% non-WT cases include congenital mesoblastic nephroma (18%), malignant rhabdoid rumor (8%), clear cell sarcoma of kidney (2%), and tumors of unknown histological type (6%) 3,4 . The average onset age of WT is 38 months, which is 6 months older in female patients than in male patients 3,5 . ...
... Relevant multi-center trials uncovered that in kidney neoplasm patients younger than 7 months old, about 66% cases are WT, and the remaining 34% non-WT cases include congenital mesoblastic nephroma (18%), malignant rhabdoid rumor (8%), clear cell sarcoma of kidney (2%), and tumors of unknown histological type (6%) 3,4 . The average onset age of WT is 38 months, which is 6 months older in female patients than in male patients 3,5 . Morphologically, WT is similar to that of embryonic kidney with structural disorders. ...
Objective:
To uncover the potential influence of microRNA-203a-5p (miRNA-203a-5p) on the malignant progression of Wilms' tumor (WT).
Patients and methods:
MiRNA-203a-5p levels in 49 paired WT and paracancerous tissues were determined by quantitative Real Time-Polymerase Chain Reaction (qRT-PCR). Prognostic value of miRNA-203a-5p in WT was assessed by the Kaplan-Meier method. Correlation between miRNA-203a-5p level and clinical data of WT patients was analyzed. In G401 and SK-NEP-1 cells, in vitro functions of miRNA-203a-5p in regulating metastatic abilities were explored. The interaction between miRNA-203a-5p and JAG1, and their regulatory role in the malignant progression of WT were evaluated by Dual-Luciferase reporter gene assay and rescue experiments.
Results:
MiRNA-203a-5p was downregulated in WT tissues than that of paracancerous ones. WT patients expressing low level of miRNA-203a-5p had higher risk of lymphatic metastasis and worse prognosis. Overexpression of miRNA-203a-5p attenuated migratory and invasive abilities in G401 cells. On the contrary, knockdown of miRNA-203a-5p yielded the opposite trends in SK-NEP-1 cells. JAG1 was verified to be the direct gene binding miRNA-203a-5p, which was negatively regulated by miRNA-203a-5p in WT cells. Rescue experiments finally uncovered that miRNA-203a-5p alleviated the malignant progression of WT via negatively regulating JAG1.
Conclusions:
MiRNA-203a-5p is downregulated in WT and closely linked to lymphatic metastasis of WT patients. By negatively regulating JAG1, miRNA-203a-5p alleviates the malignant progression of WT.
... As with the other tumors discussed in this review, Wilms tumor occurs mainly in young children, with the peak age at diagnosis of 3-4 years. Bilateral disease is seen in approximately 5% of patients and Wilms tumor is associated with genitourinary abnormalities and other syndromes such as Beckwith-Wiedemann syndrome and Denys-Drash syndrome [31]. The presenting symptom is typically a painless, palpable abdominal mass, and this is seen in approximately 80% of the patients. ...
Most pediatric malignancies require some form of cross-sectional imaging, either for staging or response assessment. The majority of these are solid tumors and this review addresses the role of MRI, as well as other cross-sectional and functional imaging techniques, for evaluating the most common pediatric solid tumors. The primary emphasis is on neuroblastoma, hepatoblastoma and Wilms tumor, three of the most common non-central-nervous-system (CNS) pediatric solid tumors encountered in young children. The initial focus will be a review of the imaging techniques and approaches used for diagnosis, staging and early post-treatment response assessment, followed by a discussion of the role surveillance imaging plays in pediatric oncology and a brief review of other emerging imaging techniques. The lessons learned here can be applied to most other pediatric tumors, including rhabdomyosarcoma, Ewing sarcoma and osteosarcoma, as well as germ cell tumors, neurofibromatosis and other rare tumors. Although lymphoma, in particular Hodgkin lymphoma, represents one of the more common pediatric malignancies, this is not discussed in detail here. Rather, many of the lessons that we have learned from lymphoma, specifically with regard to how we integrate both anatomical imaging and functional imaging techniques, is applied to the discussion of the other pediatric solid tumors.
