Overexpressed nephroblastoma protein - a review (2023)

Conversely, CCN3 protein inhibits VSMC proliferation in a TGF-β-independent manner by partially increasing CDKI p21 through Notch signaling and thereby suppressing neointimal thickening.

background:Vascular Medicine: A Companion to Braunwald's Heart Disease (Second Edition), 2013

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Extracellular matrix and eggshells

Josephine C. Adams, emCurrent issues in developmental biology, 2018

2.1 CCN-Protein

CCN proteins (for CYR61/CTGF/NOV) were initially identified in multiple cellular contexts and studied for their growth-promoting activities. Six CCN proteins are now known to exist in mammals. All are short polypeptides (281–380aa) comprising four major domains, with the exception of CCN5, which lacks a cysteine ​​junction domain (illustration 1). CCNs have multiple "parent" domains, which are proteins that share one or two domains with CCNs. CCNs are related to the IGFBP family through the N-terminal domain of the insulin-like growth factor-binding protein (IGFBP) (Vilmos, Gaudenz, Hegedus & Marsh, 2001). The domain organization of a von Willebrand factor_C (vWF_C) domain followed by a TSR domain also occurs in some TSPs (illustration 1). Although initially recognized only in vertebrates, CCN-like proteins are also encoded in Amphioxus.Branchiostoma floridaeand the urochordateciona intestinalis(Mosher & Adams, 2012). Indeed, like TSPs, CCNs are “CIBLIN” genes: conserved in bilaterians and lost in nematodes (Erives, 2015). A single CCN is encoded in annelids and many species of arthropods and molluscs (e.g.DrosophilaCCN, GenBankNP_730294). However, no CCN-like proteins have been identified in cnidarians or sponges, suggesting that CCNs arose in the last common ancestor of the two animals.

Overexpressed nephroblastoma protein - a review (1)

illustration 1. Domain architectures of matricellular proteins and some prominent ECM domain relatives. Unless otherwise noted, diagrams are based on human proteins. Domains were identified by CCD and InterProScan and coils were identified by MARCOIL. Not to scale: Tenascin-X contains 32 FNIII domains.m, Hand E/F;EGF, epidermal growth factor-like domain.tb, transforming growth factor β binding domain.TSR, thrombospondin type 1.

(Video) Neuroblastoma: Osmosis Study Video

Taken together, mammalian CCNs play multiple developmental roles in cartilage, bone, and other organs. They promote angiogenesis and are elevated in many disease processes, including wound healing, inflammation, fibrosis, and cancer.Hutchenreuther, Leask & Thompson, 2017;Krupska, Bruford and Chaqour, 2015). In particular, CCN2 has an advantageous effect as a driver of myofibroblast differentiation in various tissues.Hall-Glenn & Lyons, 2011;Leask, 2013;Maurer, 2013). Unlike the other families of mesenchymal genes, there are severalccnHowever, gene knockout mice display profound developmental phenotypesccn3mccn4Gene knockout mice are viable. For this reason,ccn1−/−Mice die during embryogenesis due to failure of vascular development resulting in hypovascularization of the placenta.Mo et al., 2002).Ctfg/ccn2−/−Mice die at birth with defective development of pancreatic islet organs, skeleton and palate and respiratory failure.Crawford et al., 2009;Ivkovic et al., 2003;Lampi et al., 2012;Tarr et al., 2017).Cnn5−/−Mice die during embryogenesis during germline implantation (Myers, Fourth, Richey, Lem Castellot, 2012). Specific knockout of the mammary glandccn6leads to an increased incidence of invasive breast carcinomas.Martin et al., 2017). Point mutations occur in humansCCN6associated with progressive pseudorheumatoid dysplasia (Table 1).

CCNs associate with other ECM proteins and bind to growth factors, cytokines, and a variety of cell surface receptors including αβ3 and α5β1 integrins and heparan sulfate proteoglycans as integrin co-receptors.Lau, 2016). As discussed in detail byLau (2016)There are numerous additional receptors for CCN2 on different cells, including insulin-like growth factor (IGF)-2 receptor, LDL receptor-related protein-1 (LRP-1), TrkA, growth factor receptor-2 fibroblasts and factor κB receptor activator and specific dendritic cell transmembrane protein. Notch-1 has been identified as a receptor for CCN3 (Wagener, Yang, Kazuschke, Winterhager & Gelhaus, 2013).

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Interaction between inflammation and extracellular matrix after myocardial infarction

Yonggang Ma, ... Merry L. Lindsey, emInflammation in heart failure, 2015

4.5.6 CCN

The CCN family consists of cysteine-rich protein 61 (CCN1), connective tissue growth factor (CTGF, CCN2),overexpressed nephroblastoma protein(CCN3) and the Wnt-inducible secreted proteins CCN4, CCN5 and CCN6. CCN proteins regulate cell adhesion, migration and survival. CCN1, CCN2 and CCN3 promote the migration of inflammatory cells, endothelial cells and fibroblasts, while CCN4 and CCN5 inhibit their migration.71,72]. CCN1 induces the proinflammatory polarization of macrophages and promotes the expression of genes responsible for adhesion, angiogenesis, and ECM renewal.73]. CCN1 is strongly induced in end-stage ischemic cardiomyopathy and regulates apoptosis, angiogenesis, inflammation and fibrotic response.74,75]. In an ischemia reperfusion model, overexpression of CCN2 in cardiomyocytes reduces infarct size by modulating Akt/p70S6/GSK-3β kinase signaling [76].in vitro, CCN4 stimulates cardiomyocyte hypertrophy, inhibits cardiomyocyte apoptosis, and facilitates cardiac fibroblast proliferation.77]. After myocardial infarction, CCN4 induction peaks at the infarct tip and distal regions at 24 h [77]. Actions related to MI by other CCN members have not been investigated.

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(Video) Nephroblastoma (Wilm's tumor)

In vitro models of vitiligo

Muriel Cario-André, ... Julien Seneschal, emskin tissue models, 2018

2.3 Genetic modification of cells

Because it is difficult to obtain enough cells from vitiligo patients to test all hypotheses, genetic data suggest that we can modify sensitive genes using overexpression or silencing vectors, such as nephroblastoma overexpression protein (NOV), also known as CCN3 (homeostasis ). melanocyte adhesion), discoidin receptor 1 (DDR1) (melanocyte adhesion to basement membrane) and cadherins (cell adhesion)[20,21]. Depending on the time of experiment or laboratory agreement, two types of vectors can be used in the cells. For a short time, transient modification (transfection) studies are performed using a plasmid encoding the gene of interest or siRNA.[20]. However, long-term studies require viral infection (transduction) using viral particles. Different types of viruses encoding the gene of interest or shRNA can be used. Viruses (retroviruses, lentiviruses, adenoviruses, or adenoviruses (AAV)) differ primarily in their ability to infect proliferating or quiescent cells and to integrate or not into the host genome. To circumvent the fact that shRNA does not cause 100% inhibition, CRISPR technology was developed. Another important point, especially in long-term studies, is the decision whether to express or suppress the gene permanently or at a specific point in time. In the latter case, inducible promoters or tet-on/tet-off technologies are used.

After the first or second passage, cells are incubated with virus particles at a multiplicity of infection (MOI) of 10 in a small volume of serum-free medium. After 6-16 hours, fresh medium containing FCS is added to bring the volume to normal (eg to 25 cm).2Transduction takes place in 0.5 ml of serum-free medium and then 3 ml of medium containing FCS is added. The medium is changed after 48 h and the percentage of transduction using a fluorescent reporter gene (eg, RFP, GFP, and YFP) present in the vector is estimated 72 h after transduction to avoid overestimation due to pseudoconversion. To achieve 100% transduction, an antibiotic resistance gene, such as the puromycin resistance gene, can be added to the vector construct. Alternatively, cells can be sorted by flow cytometry to achieve approximately 95% transduction.

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(Video) Wilms Tumour Webinar

Hematopoietic Stem Cells

Malcolm A. S. Moore, emPrinciples of Tissue Engineering (Fourth Edition), 2014

Genes associated with CHS

Aldehyde dehydrogenase (ALDH) has been reported to be a key regulator of HSC differentiation[33]. Inhibition of ALDH with diethylaminobenzaldehyde (DEAB) delayed the differentiation of human HSCs that would otherwise occur in response to cytokines. In addition, short-term culture with DEAB induced a 3.4-fold increase in HSC[33]. The effects of DEAB on HSC differentiation can be reversed by co-administration of the retinoic acid receptor agonist all-trans retinoic acid, suggesting that the ability of ALDH to produce retinoic acids is important in determining the fate of HSCs. High levels of the ALDH1A1 isoform of aldehyde dehydrogenase are expressed in HSCs, but loss of ALDH1A1 does not affect them[34]. ALDH1A1 deficiency is associated with increased expression of the ALDH3A1 isoform, suggesting that it may balance ALDH1A1. ALDH3A1 and ALDH1A1-deficient mice exhibit reduced HSC numbers and abnormal cell cycle distribution, increased levels of reactive oxygen species, p38 mitogen-activated protein kinase activity, and susceptibility to DNA damage[34]. These results demonstrate that ALDH3A1 can compensate for ALDH1A1 and that both ALDH1A1 and 3A1 genes are important in HSC metabolism of reactive oxygen species and reactive aldehydes.

Matricellular protein Nephroblastoma Overexpressed (Nov, CCN3) is essential for HSC functional integrity[35]. Forced expression of Nov or addition of recombinant Nov protein increases HSC and/or progenitor activity. Scl and Lyl1 encode two related basic helix-loop-helix transcription factors, and single- and double-knockout studies in mice have shown that the expression of at least one of these factors is necessary to maintain HSC function in adults[36]. Lineage-related transcription factors must be tightly regulated in HSC self-renewal and commitment decisions. During embryogenesis, Runx1/AML1 and Evi-1 are essential for HSCs, while in adults Runx1 and Evi-1 up- and down-regulate HSCs, respectively (see ref.).[37]). The tumor suppressor and cell contact inhibitory mediator Nf2/Merlin is critical for maintaining the normal structure and function of the HSC niche[38]. HSCs in Nf2-deficient mice were increased in number and exhibited a marked change in circulatory location, which was associated with increases in vascular endothelial growth factor (VEGF) and bone and vascular components[38]. Nf2 demarcates the bony and vascular components of the niche and thus restricts HSC number and location. Disruption of the c-myb proto-oncogene specifically in the adult bone marrow resulted in critical depletion of the HSC pool with impaired hematopoiesis and a severe reduction in neutrophil, monocyte, B-lymphoid, erythroid, and megakaryocytic cell numbers[39]. This transcription factor is an essential regulator of self-renewal and multilineage differentiation of long- and short-term adult HSCs and pluripotent progenitors. Co-expression of lymphoid and myeloid genes is an early event that occurs in pluripotent progenitors but also in approximately 30% of short-lived HSCs[40].

Proliferative nuclear antigen-associated factor (PAF) is highly expressed in the BM-HSC cycle and plays a key role as a key regulator of early hematopoiesis[41]. Mice lacking Paf showed reduced BM cellularity and leukopenia with reduced numbers of HSCs and impaired progenitors. These phenotypes are caused by an intrinsic cell block in the development of long-term HSCs (LT) into pluripotent progenitors and a preferential loss of lymphoid progenitors caused by significantly increased p53-mediated apoptosis[41]. In addition, LT-HSCs from Paf(−/−) mice displayed increased levels of reactive oxygen species (ROS), failed to maintain quiescence, and were unable to support long-term hematopoiesis.

Focal adhesion kinase (Fak) is a receptor-free protein tyrosine kinase that plays an essential role in many cell types, where its activation controls adhesion, motility and survival. Fak expression is relatively elevated in HSCs compared to progenitor cells and mature blood cells[42]. Extensive Fak inactivation in the hematopoietic system leads to an increase in the number of activated HSCs, likely due to changes in the mutual interactions between HSCs and their microenvironment[42]. It has been reported that the endoplasmic reticulum chaperone protein GRP94 is essential for the expression of specific integrins and is required to maintain HSC interactions with the BM site in adults[43]. There was an increase in HSCsand granulocyte-monocyte precursors in Grp94−/−BM is associated with loss of HSC quiescence and increased proliferation. This expansion of the HSC pool may be attributed to the impaired interaction of HSCs with their niche, indicated by severely reduced HSC homing and homing potential, as well as increased mobilization[43].

Endoglin is an accessory transforming growth factor-beta (TGF-beta) receptor that has recently been shown to be highly expressed in long-term repopulated HSCs[44]. However, little is known about its function in these cells. Transplantation of BM enriched with HSCs and progenitor cells revealed that neither endoglin suppression nor endoglin overexpression affected the ability of stem cells to repopulate recipient marrow in the short or long term[44]. Reduced endoglin expression increased the clonogenic capacity of erythroid precursors of the blastocyst, while overexpression reduced erythroid differentiation to the basophilic erythroblast phase, suggesting that endoglin plays a key role in key stages of adult erythropoietic development[44].

Protein regulation through the ubiquitin-proteasome system (UPS) has recently been shown to be critical for normal HSC function (see ref.).[26]). Casita B-cell lymphoma (c-Cbl), Itch and Fbw-7 reported as the 'sleeping guardians' of the HSC population[26]. The c-Cbl proto-oncogene is an E3-finger ubiquitin ligase that is the cellular homologue of v-Cbl, a retroviral transformant c-Cbl gene that directly or indirectly regulates approximately 150 proteins and regulates Notch1 activity downregulates , c- Kit and Signal Transducer and Transcriptional Activator (STAT)5 (activator of c-myc expression), which contribute to the maintenance of HSC[26]. Cbl knockout mice show cell-autonomous growth in HSCs without an increase in mature cell production. cbl−/−HSCs show increased levels of phospho-STAT5 and Myc mRNA, suggesting that Cbl deficiency stabilizes active STAT5. c-Cbl-E3 ligase activity keeps HSC proliferative capacity in check, with loss of activity leading to increased self-renewal and abnormal proliferation in a normally quiescent adult HSC compartment[26]. Itch belongs to the HECT family of ligases (E6AP C-terminal homolog) and, similar to c-Cbl, functions as a negative regulator of HSC self-renewal and proliferation through Notch, a known regulator of hematopoiesis[26]. Fbw-7 is an E3 finger RING ligase that plays a role in the regulation of about 20 proteins including c-myc and Notch. Germline deletion of Fbw7 leads to embryonic lethality around E10.5 due to vascular defects resulting from stabilization of Notch4 in the embryo. Conditional deletion of Fbw7 leads to an increase in the frequency of active cycles of HSCs with their eventual loss. Fbw7−/−HSC defects are cell-autonomous and include downregulation of genes involved in HSC quiescence, with an overall loss of quiescence characteristics[26].

USP1 is a DUB cysteine ​​protease that deubiquitinates FANCD2 (Faconi anemia group D2), a regulator of Faconi anemia (FA) signaling. It is able to protect HSCs from DNA damage during the divisions required to maintain the stem cell pool (see ref).[26]). RelB and nuclear factor κB (NF-κB2), major effectors of aberrant NF-κB signaling, intrinsically up-regulate HSC and renew progenitor cells[45].

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(Video) Pathology 700 a Wilms Tumor Kidney Nephroblastoma embryonal WAGR syndrome microscopy cancer child


TGF beta -1, -2 and -3 in the regulation of fibrosis in the cornea and other organs

Steven E. Wilson, εμExperimental ophthalmological research, 2021

4.1 Connective Tissue Growth Factor (CTGF)

CTGF is a mitogenic heparin-binding protein secreted by fibroblasts and other connective tissue cells following stimulation with TGF beta (Grotendorst, 1997;Ramazani et al., 2018). It is a member of the CCN family of regulatory proteins that modulate the ECM and share structurally related functional domains—such as cysteine-rich angiogenic inducer 61 (CYR61);overexpressed nephroblastoma protein(NOV) and WNT1-induced signaling pathway proteins WISP1, WISP2 and WISP3. CTGF is a downstream mediator of TGF-beta that stimulates connective tissue cell proliferation and ECM synthesis. It has been reported not to modulate epithelial or immune cells (Grotendorst, 1997;Ramazani et al., 2018).

Most CCN family members share a similar structure with four conserved domains: 1) an insulin-like growth factor-binding protein domain, 2) a von Willebrand factor C repeat, 3) a CCN family repeat. thrombospondin types 1 and 4) a cysteine ​​knot (Grotendorst, 1997;Ramazani et al., 2018). The hinge between domains 2 and 3 can be cleaved by various proteases. Each domain has been shown to have specific biological functions, but they also work together in crosstalk between different signaling pathways. CTGF binds structural components of the ECM important for TGF-beta signaling, including perlecan and decorin, discussed later in this review, with integral cell surface molecules such as integrins and connexins 43 (June and Lau, 2011;Aguiar et al., 2014).

Transcription of CTGF mRNA is induced by many stimuli, including growth factors and cytokines, hormones, mitogens, mechanical stress, UV light, and oxygen deprivation.Kubota and Takigawa, 2015). Most TGFβ-mediated responses involve stimulation of CTGF at some level, including stimulation of ECM component production, fibroblast proliferation, and other processes during wound healing and fibrosis.Xue et al., 2015;Cho and others, 2015), but there is often interaction between the stimuli. For example, the synergy between hypoxia and TGF-β signaling increases CTGF expression in muscle fibers but not in fibroblasts (Valle-Tenney et al., 2020). In other pathophysiological processes, CTGF appears to function independently of TGF-beta. For example, CTGF is not upregulated by normal TGF-β stimulation shortly after skeletal muscle denervation, and other factors are likely involved in fibrotic responses in these tissues.Rebolledo et al., 2019). Many miRNAs are involved in the regulation of CTGF expression.Ramazani et al., 2018).

Some attention has been paid to the potential differential effects of TGF beta-1. -2 or -3 in CTGF mRNA expression. In a mouse model study of anti-thy 1.1 glomerulonephritis (Ito et al., 2001), while differential expression of the three TGF-beta isoforms emerged over timeno partAfter injury, all three isoforms were found to be equivalent in regulating CTGF mRNA in cultured mesangial cells and glomerular epithelial cellsin vitro. In another study in which primary human fibroblasts were isolated in culture from hypertrophic scars, keloid or normal skin (Colwell et al., 2005) increased CTGF expression in normal fibroblasts more than 150-fold when stimulated with TGF beta-1, but also more than 100-fold when stimulated with TGF beta-2 or TGF beta-3 stimulated. CTGF expression was higher after TGF-beta-1 stimulation in hypertrophic circular fibroblasts compared to TGF-beta-2 and TGF-beta-3, however, there was still significant upregulation of CTGF by all three TGF -beta-isoforms. CTGF expression of keloid fibroblasts was also increased more than 100-fold after stimulation with TGF beta-1 and even more than 75-fold after addition of TGF beta-2 or TGF beta-3. Based on the hypothesis of an antifibrotic role of TGF beta-3, one would expect that TGF beta-3 would reduce CTGF expression, but this does not appear to be the case. However, this needs to be studied in other tissues before any firm conclusions can be drawn about it.

CTGF contributes significantly to renal, cardiovascular and skeletal development, with TGF-beta also heavily involved.Ramazani et al., 2018). It is also involved in the development of fibrosis in most organs, including the heart, kidney, liver, lung, skin and eyes, where TGF beta plays an important role (Ramazani et al., 2018). It is also involved in TGF-beta-related carcinogenesis and tumor growth.Wells et al., 2015). Therefore, CTGF also plays a role in many processes in which TGF-beta plays an important role.

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What are the symptoms of nephroblastoma? ›

Children with Wilms tumor (also known as nephroblastoma) usually have a swelling or bump in their belly. Often, there are no other symptoms. But some children may also have: nausea.

What is the most important prognostic marker of Wilms tumor? ›

Age. Younger age is a favourable prognostic factor. Children under 2 with favourable histology tumours that have not spread have a better prognosis than older children.

What is the difference between a Wilms tumor and a nephroblastoma? ›

Neuroblastoma (NBL) is the most common extra-cranial tumour in childhood [1] and commonly presents as an abdominal mass. Nephroblastoma, also more commonly known as a Wilms' tumour, is the commonest renal tumour in childhood and also typically presents as abdominal pathology.

What are the risk factors for nephroblastoma? ›

Children with certain genetic and overgrowth syndromes, such as WAGR (Wilm's tumour, Aniridia, Genitourinary malformations, and Retardation), Denys-Drash and Beckwith-Wiedemann, are at an increased risk of nephroblastoma.

Is nephroblastoma a malignant tumor? ›

Wilms tumor, also called nephroblastoma, is a malignant (cancerous) tumor originating in the cells of the kidney. It is the most common type of renal (kidney) cancer and accounts for about 6 percent of all childhood cancers.

What is the prognosis of nephroblastoma? ›

Treatment involves bilateral renal biopsy, nephrectomy, chemotherapy and radiotherapy. The 4-year survival rate ranges from 42% to 80%, depending on degree of anaplasia.

What is an unfavorable Wilms tumor? ›

"Unfavorable" Histology: Wilms tumors with "unfavorable" histology will demonstrate much higher degrees of anaplasia and are associated with a relatively poorer prognosis and survival.

What is stage 4 Wilms tumor? ›

Stage IV: Cancer has spread to other, more distant organs, such as the lungs, liver, bones, and brain, or to lymph nodes outside the abdomen. This is called metastasis. Stage V: Cancer cells are in both kidneys at the same time. The tumor in each kidney is staged separately.

How do you treat Stage 3 Wilms tumor? ›

Treatment for stage 3 Wilms tumour is usually chemotherapy for 4 weeks. Then surgery to remove the kidney. After surgery, your child will usually have chemotherapy. For low and medium risk tumours they have 27 weeks of chemotherapy.

What is the most accurate type of diagnosis for Wilms tumor? ›

For most types of tumors, a biopsy is the only sure way for the doctor to know if cancer is present. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

What is the most common feature of Wilms tumor? ›

The most common symptom is a swollen abdomen, which is usually painless. Sometimes a parent or carer may feel a lump in the abdomen which can be quite large. Occasionally, the tumour may bleed slightly and this can irritate the kidney and may be painful.

What is the common metastasis of Wilms tumor? ›

The most common site of metastatic spread of Wilms tumor is the lungs; chest CT scan is the preferred imaging modality for evaluating this site. Other rare sites of metastases, such as the liver, are usually well evaluated with the initial abdominopelvic CT scan.

What are the 3 syndrome associated with Wilms tumor? ›

The most common syndromes associated with WT are WAGR (Wilms-Aniridia-Genitourinary-mental Retardation), Denys-Drash syndrome (DDS), Beckwith-Wiedemann syndrome (BWS), isolated hemihypertrophy, and Perlman syndrome.

What is the overall survival of Wilms tumor? ›

One-year overall survival was found to be 59.3% (95% CI: 40.7–73.3), with tumor size greater than 15 cm (p 0.021) and unfavorable WT type (p 0.012) being the predominant predictors.

What is an important intervention to take for a child with nephroblastoma? ›

Nursing Interventions

Monitor I.V. fluid therapy and intake and output carefully, including nasogastric (NG) drainage. Encourage the parents to ask questions and to understand fully the risk and benefits of surgery. Prepare the child for surgery, explain the procedures at the appropriate developmental level.

Is Wilms tumor fatal? ›

The 5-year relative survival rate for children with a Wilms tumor is 93%. The survival rates for children with a Wilms tumor vary based on several factors. These include the stage of tumor, a person's age and general health, and how well the treatment plan works.

Is Wilms tumor aggressive? ›

But they soon found out her symptoms were caused by something much worse: a Wilms tumor (a type of kidney tumor) with diffuse anaplasia (this means cells that are aggressive and resistant to treatment). This is the rarest type of Wilms tumor and the most challenging to overcome.

How do you treat Stage 5 Wilms tumor? ›

Chemotherapy is given to shrink the tumours, and then surgery is done. The goal of surgery is to remove as much of the tumour from each kidney as possible while leaving enough normal kidney tissue behind. Chemotherapy is usually given again after surgery. Radiation therapy may also be given after surgery.

What is the rule of 10 in nephroblastoma? ›

Wilms tumour classically follows the “rule of 10's”: up to 10% may have unfavourable histology, 10% are bilateral, 10% have vascular invasion, 10% have calcifications on CT and 10% have pulmonary metastases at presentation [19].

Can you remove a Wilms tumor? ›

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. Surgery is a common treatment for all stages of a Wilms tumor. It is important that your child's surgeon has experience with removing Wilms tumors and, in some cases, experience with saving part of the kidney.

What are the long term effects of Wilms tumor? ›

Depending on their age when they were diagnosed and treated for a Wilms tumor, a child may experience dental issues, including problems with teeth formation, enamel issues, or missing teeth.

Does Wilms tumor need chemo? ›

Wilms tumour is curable in more than 9 out of 10 children (90%). The main treatments include: chemotherapy for almost all children. surgery for all children.

Is Wilms tumor leukemia? ›

Wilms' tumor gene 1 is an independent prognostic factor for pediatric acute myeloid leukemia following allogeneic hematopoietic stem cell transplantation.

Does Wilms tumor metastasize? ›

The tumor can be very large before it's found. And it may spread (metastasize) to other body tissues. The most common site for Wilms tumor to spread to is the lungs. But it may also spread to the liver, lymph nodes, other kidney, brain, and bones.

Can a Wilms tumor burst? ›

Wilms tumor rupture is rare, constituting 3% of all the cases of Wilms tumor (1,2). Preoperative rupture is rarer and could either be post-traumatic or spontaneous in nature (1,2). Diagnosis of preoperative rupture is based on clinico-radiological signs (1,3).

What is the average age of Wilms tumor? ›

Wilms tumor (WT) is the most common type of childhood renal cancer. It affects approximately one child per 10,000 worldwide before the age of 15 years (1). The median age at diagnosis of WT is approximately 3.5 years (1).

Can Wilms tumor be cured without surgery? ›

Treatment for Wilms tumor usually involves surgery and chemotherapy. It sometimes includes radiation therapy. Treatments depend on the stage of the cancer. Because this type of cancer is rare, a children's cancer center that has treated this type of cancer might be a good choice.

What are the late effects of treatment for Wilms tumor? ›

Kidney failure can be a late effect for children who have Wilms tumour in both kidneys. Kidney problems can develop 10 to 20 years after a nephrectomy, radiation therapy or chemotherapy to treat Wilms tumour. Depending on the dose, radiation therapy can cause kidney damage (called radiation nephritis).

What is the survival rate for Stage 4 Wilms tumor? ›

The overall cure rate is approximately 85%, with about 90% of Stage I, 99% of Stage II, 85% of Stage III, 66% of Stage IV, and 66% of unfavorable histology (UH) patients cured. Patients with initial Stage I or Stage II Wilms Tumor who relapse can still be cured using more intense chemotherapy.

What is a tumor marker for a Wilms tumor? ›

A tumor marker, also called a biomarker, is a substance found in a person's blood, urine, or body tissue at a level that indicates a possible disease. Researchers have found that Wilms tumors with a favorable histology and changes in chromosome 1q are more likely to come back after treatment.

How do you treat Stage 2 Wilms tumor? ›

Surgery is the main treatment for stage 2 Wilms tumour. It is followed by radiation therapy or chemotherapy or both, depending on tumour histology. The most common surgery used is a radical nephrectomy (removal of the kidney) with lymph node removal.

Is nephroblastoma benign or malignant? ›

About Wilms tumor

It can also be called a nephroblastoma. A Wilms tumor is always cancerous. It is the most common type of kidney cancer diagnosed in children. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body.

What is the serious potential complication of Wilms tumor? ›

Spread of the tumor to the lungs, lymph nodes, liver, bone, or brain is the most worrisome complication. High blood pressure and kidney damage may occur as the result of the tumor or its treatment.

What are the neurological symptoms of Wilms tumor? ›

Neurologic issues: Nervous center issues include seizures, brain malformations, or muscle weakness/tightness. End-stage renal (kidney) disease: Renal failure may develop in patients over time. This feature is generally worse in patients who have been treated for a Wilms tumor.

What is the prognosis for Wilms tumor in adults? ›

Wilms tumor is rare in adulthood. The prognosis is poor when compared with children. Adults more frequently present with advanced disease stages, and chemotherapy has a moderate effect.

What are the stages of Wilms tumor? ›

Based on the results of the imaging studies and surgery, a clinical stage will be assigned:
  • Stage I tumor completely removed.
  • Stage II tumor grew through the kidney capsule but was completely removed.
  • Stage III residual abdominal tumor (due to lymph node spread, spillage of. ...
  • Stage IV tumor spread to the lung or the liver.

What does Wilms tumor do to the body? ›

The cancer cells live while healthy cells die as part of their natural life cycle. With Wilms tumor, the changes make extra cells in the kidney that form the tumor. Rarely, DNA changes passed from parents to children can increase the risk of Wilms tumor.

What is the most common presenting feature of Wilms tumor? ›

The most common manifestation of Wilms tumor is an asymptomatic abdominal mass; an abdominal mass occurs in 80% of children at presentation. Abdominal pain or hematuria occurs in 25%.

At what age does nephroblastoma occur? ›

Wilms tumor, or nephroblastoma, is the most common renal cancer in the pediatric age group. [1][2] It is also the most common pediatric abdominal cancer and the fourth most common pediatric cancer overall. Wilms tumor is typically found in children younger than five years old.

Which of the following is a common presenting symptom for Wilms tumor? ›

Signs and symptoms of Wilms tumor include: A swollen spot or hard lump in your child's abdomen (stomach area). The lump or swelling can be painful, but it's usually not. Pain in their abdomen.

Is a nephroblastoma benign or malignant? ›

About Wilms tumor

It can also be called a nephroblastoma. A Wilms tumor is always cancerous. It is the most common type of kidney cancer diagnosed in children. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body.

What is the classic triad of Wilms tumor? ›

Wilms' tumour (WT) is the most common paediatric renal tumour, which can present as a single nodule, as multifocal unilateral lesions or as bilateral tumours. Typically, WT comprises three histological components namely blastemal, epithelial and stromal.

How do you treat Stage 4 Wilms tumor? ›

Treatment for stage 4 Wilms tumour is usually chemotherapy for 6 weeks, then surgery to remove the kidney. After surgery, your child is likely to have more chemotherapy and may have radiotherapy.

What is the first site of metastasis of Wilms tumor? ›

The most common site of metastatic spread of Wilms tumor is the lungs; chest CT scan is the preferred imaging modality for evaluating this site. Other rare sites of metastases, such as the liver, are usually well evaluated with the initial abdominopelvic CT scan.


1. Aging and activating the heat shock response.
(The Sheekey Science Show)
2. The Role of Partial Nephrectomy in the Management of Wilms Tumor
(Breakthroughs for Physicians)
3. The Stem Cell Regulator Lin28 in Malignancy
(The Society for Translational Oncology)
4. Wilms' tumor detection, treatment and research
(MD Anderson Cancer Center)
5. wilms tumor part 8
6. Nephroblastoma
(General Surgery - Topic)


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Introduction: My name is Allyn Kozey, I am a outstanding, colorful, adventurous, encouraging, zealous, tender, helpful person who loves writing and wants to share my knowledge and understanding with you.