Tumor pediátrico embrionário do rim que também pode ser observado raramente em adultos. O pico de incidência do tumor de Wilms ocorre entre o segundo e o quinto ano de vida. Microscopicamente, é composto por uma mistura de elementos celulares (blastemal, estromal e epitelial). Os locais mais comuns de metástase incluem os linfonodos regionais, pulmões e fígado.
Introdução
O que você precisa saber de cara
Tumor pediátrico embrionário do rim que também pode ser observado raramente em adultos. O pico de incidência do tumor de Wilms ocorre entre o segundo e o quinto ano de vida. Microscopicamente, é composto por uma mistura de elementos celulares (blastemal, estromal e epitelial). Os locais mais comuns de metástase incluem os linfonodos regionais, pulmões e fígado.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 13 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 28 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
10 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Not applicable.
Component of the PAF1 complex (PAF1C) which has multiple functions during transcription by RNA polymerase II and is implicated in regulation of development and maintenance of embryonic stem cell pluripotency. PAF1C associates with RNA polymerase II through interaction with POLR2A CTD non-phosphorylated and 'Ser-2'- and 'Ser-5'-phosphorylated forms and is involved in transcriptional elongation, acting both independently and synergistically with TCEA1 and in cooperation with the DSIF complex and H
Nucleus speckle
Involved in double-strand break repair and/or homologous recombination. Binds RAD51 and potentiates recombinational DNA repair by promoting assembly of RAD51 onto single-stranded DNA (ssDNA). Acts by targeting RAD51 to ssDNA over double-stranded DNA, enabling RAD51 to displace replication protein-A (RPA) from ssDNA and stabilizing RAD51-ssDNA filaments by blocking ATP hydrolysis. Part of a PALB2-scaffolded HR complex containing RAD51C and which is thought to play a role in DNA repair by HR. May
NucleusCytoplasm, cytoskeleton, microtubule organizing center, centrosome
Breast cancer
A common malignancy originating from breast epithelial tissue. Breast neoplasms can be distinguished by their histologic pattern. Invasive ductal carcinoma is by far the most common type. Breast cancer is etiologically and genetically heterogeneous. Important genetic factors have been indicated by familial occurrence and bilateral involvement. Mutations at more than one locus can be involved in different families or even in the same case.
Nuclear corepressor for KRAB domain-containing zinc finger proteins (KRAB-ZFPs). Mediates gene silencing by recruiting CHD3, a subunit of the nucleosome remodeling and deacetylation (NuRD) complex, and SETDB1 (which specifically methylates histone H3 at 'Lys-9' (H3K9me)) to the promoter regions of KRAB target genes. Enhances transcriptional repression by coordinating the increase in H3K9me, the decrease in histone H3 'Lys-9 and 'Lys-14' acetylation (H3K9ac and H3K14ac, respectively) and the disp
Nucleus
Wilms tumor 7
A pediatric malignancy of kidney, and the most common childhood abdominal malignancy. It is caused by the uncontrolled multiplication of renal stem, stromal, and epithelial cells.
Probable transcription factor likely to be involved in early steps in the differentiation of amacrine and ganglion cells. Recognizes and binds to the DNA sequence 5'-ATGCAAAT-3'. Isoform 1 does not bind DNA
Nucleus
Hereditary susceptibility to Wilms tumor 5
Pediatric malignancy of kidney and one of the most common solid cancers in childhood.
Plays a key role in chromosome recombination and chromosome structure development during meiosis. Required at early steps in meiotic recombination that leads to non-crossovers pathways. Also needed for efficient completion of homologous synapsis by influencing crossover distribution along the chromosomes affecting both crossovers and non-crossovers pathways. Also required for development of higher-order chromosome structures and is needed for synaptonemal-complex formation. In males, required fo
Mosaic variegated aneuploidy syndrome 3
A form of mosaic variegated aneuploidy syndrome, a severe disorder characterized by mosaic aneuploidies, predominantly trisomies and monosomies, involving multiple different chromosomes and tissues. Affected individuals typically present with severe intrauterine growth retardation and microcephaly. Eye anomalies, mild dysmorphism, variable developmental delay, and a broad spectrum of additional congenital abnormalities and medical conditions may also occur. The risk of malignancy is high, with rhabdomyosarcoma, Wilms tumor and leukemia reported in several cases. MVA3 inheritance is autosomal recessive.
Transcriptional repressor which binds neuron-restrictive silencer element (NRSE) and represses neuronal gene transcription in non-neuronal cells (PubMed:11741002, PubMed:11779185, PubMed:12399542, PubMed:26551668, PubMed:7697725, PubMed:7871435, PubMed:8568247). Restricts the expression of neuronal genes by associating with two distinct corepressors, SIN3A and RCOR1, which in turn recruit histone deacetylase to the promoters of REST-regulated genes (PubMed:10449787, PubMed:10734093). Mediates re
NucleusCytoplasm
Wilms tumor 6
A pediatric malignancy of kidney, and the most common childhood abdominal malignancy. It is caused by the uncontrolled multiplication of renal stem, stromal, and epithelial cells.
Transcription factor that plays an important role in cellular development and cell survival (PubMed:7862533). Recognizes and binds to the DNA sequence 5'-GCG(T/G)GGGCG-3' (PubMed:17716689, PubMed:25258363, PubMed:7862533). Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. It has a tumor suppressor as well as an oncogenic role in tumor formation. Function may be isoform-specific: isoforms lacking the KTS motif may a
NucleusNucleus, nucleolusCytoplasmNucleus speckleNucleus, nucleoplasm
Frasier syndrome
Characterized by a slowly progressing nephropathy leading to renal failure in adolescence or early adulthood, male pseudohermaphroditism, and no Wilms tumor. As for histological findings of the kidneys, focal glomerular sclerosis is often observed. There is phenotypic overlap with Denys-Drash syndrome. Inheritance is autosomal dominant.
3'-5'-exoribonuclease that specifically recognizes RNAs polyuridylated at their 3' end and mediates their degradation. Component of an exosome-independent RNA degradation pathway that mediates degradation of both mRNAs and miRNAs that have been polyuridylated by a terminal uridylyltransferase, such as ZCCHC11/TUT4. Mediates degradation of cytoplasmic mRNAs that have been deadenylated and subsequently uridylated at their 3'. Mediates degradation of uridylated pre-let-7 miRNAs, contributing to the
CytoplasmCytoplasm, P-body
Perlman syndrome
An autosomal recessive congenital overgrowth syndrome. Affected children are large at birth, are hypotonic, and show organomegaly, characteristic facial dysmorphisms (inverted V-shaped upper lip, prominent forehead, deep-set eyes, broad and flat nasal bridge, and low-set ears), renal anomalies (nephromegaly and hydronephrosis), frequent neurodevelopmental delay, and high neonatal mortality. Perlman syndrome is associated with a high risk of Wilms tumor. Histologic examination of the kidneys in affected children shows frequent nephroblastomatosis, which is a precursor lesion for Wilms tumor.
Cell surface proteoglycan (PubMed:14610063). Negatively regulates the hedgehog signaling pathway when attached via the GPI-anchor to the cell surface by competing with the hedgehog receptor PTC1 for binding to hedgehog proteins (By similarity). Binding to the hedgehog protein SHH triggers internalization of the complex by endocytosis and its subsequent lysosomal degradation (By similarity). Positively regulates the canonical Wnt signaling pathway by binding to the Wnt receptor Frizzled and stimu
Cell membrane
Simpson-Golabi-Behmel syndrome 1
A condition characterized by pre- and postnatal overgrowth (gigantism), facial dysmorphism and a variety of inconstant visceral and skeletal malformations. Characteristic dysmorphic features include macrocephaly with coarse, distinctive facies with a large protruding jaw, broad nasal bridge and cleft palate. Cardiac defects are frequent.
Variantes genéticas (ClinVar)
2,172 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 14 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
49 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Nefroblastoma
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Outros ensaios clínicos
254 ensaios clínicos encontrados, 3 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 717
International Society of Paediatric Surgical Oncology (IPSO) Minimally Invasive Surgery (MIS) Guidelines.
Paediatric oncology surgeons play a crucial role in diagnosing, staging, and treating malignant solid tumors. In recent years, many solid tumour protocols have advocated for a more tailored surgical approach to both the primary tumour site and metastatic disease. The integration of Minimally Invasive Surgery (MIS) into paediatric oncology practice has gained popularity over the past few decades. While the benefits of MIS are well established in non-oncologic surgery, its role in paediatric solid tumours is still evolving and, in many cases, lacks high-quality evidence. These IPSO-MIS guidelines guidelines aim to provide practical surgical recommendations for diverse clinical scenarios, addressing the needs of both High-Income Countries (HICs) and Low- and Middle-Income Countries (LMICs). The contributing authors represent both settings, ensuring a comprehensive and inclusive perspective. We hope that these guidelines will contribute to improving outcomes for children with cancer worldwide. Israel Fernandez-Pineda, IPSO Education Committee Chair Abdelhafeez H Abdelhafeez, IPSO Education Committee Member.
Differential immune infiltrates in histomorphologic Wilms tumor regions identify prognostic macrophages.
Wilms tumor (WT) is characterized by a unique ternary histology, including blastemal, epithelial, and mesenchymal elements. Although overall survival is high, relapse and metastasis affect not only high-risk but also intermediate-risk (IR) patients. We here analyzed immune cell infiltrates in relation to histomorphological tumor regions of patients treated with neo-adjuvant chemotherapy and evaluated their role for disease prognosis. We included 46 chemotherapy-treated WTs resected between 2002 and 2020, which affected 27 females and 19 males at a median age of 35.58 months. Tumor samples were re-evaluated resulting in 13 mixed, 12 regressive, 8 blastemal, 7 mesenchymal, and 6 epithelial subtypes. HALO was used for automated quantification of the immunohistochemical stainings. Immune markers abundance was highly dependent on the histomorphological region. We observed significantly lower amounts of CD4, CD8, and CD206 positive immune cells in the blastemal region, as compared to the mesenchymal region. Moreover, abundance of CD206, CD86, and CD68 positive immune cells in the mesenchymal and blastemal regions showed a significant association with prognosis and timing of relapse. In conclusion, WT displays region-specific differences in immune cell infiltration. Evaluating CD206, CD86, and CD68 expression in mesenchymal and blastemal regions might be valuable for improving IR patient stratification.
Understanding the Burden of Care for Children With Pediatric Solid Tumors.
A new solid tumor diagnosis imposes substantial burdens on children and their families. Prior studies have quantified burden using direct medical costs but often exclude indirect costs, logistical stress, and life disruptions. In this study, we quantify burden of care by assessing healthcare utilization and estimating the associated logistical burden during the first year following diagnosis. We conducted a retrospective chart review of 166 children (2012-2022) treated within 1 year of an extracranial solid tumor diagnosis at a single institution. We collected data on treatment modalities, healthcare utilization, estimated travel, and job opportunity costs. Altogether, 166 children (55% male, 63% White) with median age at diagnosis 2.2 (IQR 1.1-4.8) years were included. The most common diagnoses were neuroblastoma (41%), retinoblastoma (25%), and nephroblastoma (17%). Sixty-four children (39%) received ≥3 treatment modalities and 75% required tumor resection surgery. Children cumulatively spent a median of 9 days receiving medical care (5-18) at 1 month, 22 (12-35) days at 3 months, 35 (21-68) days at 6 months, and 54 (30-106) days at 1 year. Families traveled a median one-way distance of 79 (35-136) miles. Estimated annual travel costs were $2,079 ($1129-$4267) and lost wages were $15,277 ($7477-$27,101), ∼21% of median household income. Advanced-stage disease was associated with significantly more days spent receiving medical care, longer travel, and greater lost wages. We characterize the substantial medical and financial burdens on families of children with solid tumors. Decentralized care delivery, multidisciplinary care models, and policy-level approaches such as expanded insurance coverage may reduce logistical and financial burdens.
Cognitive and Psychosocial Outcome in Children and Adolescents Treated for Extracranial Malignant Solid Tumors and Lymphomas in the RISK-N Study.
Recent studies show evidence of cognitive and psychosocial impairments and reduced quality of life (QoL) in adult survivors of childhood extracranial solid tumors and lymphomas, but limited research has addressed these issues in pediatric populations. The French RISK-N prospective study (2014-2021) evaluated 278 survivors of extracranial solid tumors or lymphomas (47% female, mean age at diagnosis and assessment: 6.2 and 11.7 years). Patients with pre-existing neurological conditions were excluded. Sociodemographic, disease-related, and treatment data were collected. Cognitive performance was assessed using Wechsler Intelligence Scales for Children (WISC-IV, WISC-V). Psychosocial outcomes included parent and/or patient-reported executive functions (Behavior Rating Inventory of Executive Function), behavior (Conner's Parent Rating Scale), QoL (Pediatric Quality of Life Inventory), fatigue (Multidimensional Fatigue Scale), and depression (Children's Depression Inventory). Information on schooling and educational/rehabilitative interventions was also recorded. Diagnoses included lymphoma (25%), nephroblastoma (19%), neuroblastoma (19%), osteosarcoma (7%), other sarcomas (18%), and other tumors (12%). Mean Full Scale Intellectual Quotient [M(SD) = 99.44(15.62)] was as expected in the general population [M(SD) = 100(15)], but the WISC-IV Perceptual Reasoning Index was slightly lower [M(SD) = 95.4(15.0), <1.5 SD 14%]. Parent- and self-reports indicated greater executive dysfunction, inattention, fatigue, and reduced QoL. In multivariable regression models, poorer cognitive outcomes were associated with lower parental education and developmental/learning delays before diagnosis. Objective cognitive deficits were uncommon among pediatric cancer survivors, contrasting with a relatively high level of subjective cognitive and psychosocial complaints, highlighting the need for systematic screening and tailored clinical interventions.
Relapsed rhabdoid tumours and other non-nephroblastoma childhood and adolescent kidney tumours: perspectives from the HARMONICA collaboration.
Paediatric kidney tumours are generally associated with a favourable survival rate. Most children are diagnosed with Wilms tumour, which has a 90% long-term survival rate with conventional front-line and salvage therapies. However, treatments and outcomes of children with relapsed non-Wilms tumours, such as malignant rhabdoid tumour of the kidney, renal-cell carcinoma (including renal medullary carcinoma), clear-cell sarcoma of the kidney, anaplastic sarcoma of the kidney and congenital mesoblastic nephroma are not well defined. Several of these non-Wilms tumours include unfavourable prognostic subtypes. Currently available data on non-Wilms tumours support advancing studies that pivot from conventional strategies towards disease-specific, biologically driven novel treatments to improve outcomes for each of these rare childhood kidney tumours.
Publicações recentes
Genome-Wide Copy-Number Landscape of Germ Cell Tumors With Synchronous Conventional and "Somatic-Type" Malignancy Components.
[Wilms' tumor in a horseshoe kidney: organ-sparing treatment].
[Paediatric kidney tumours: Update on the challenges of nephroblastoma and other kidney tumours].
International Society of Paediatric Surgical Oncology (IPSO) Minimally Invasive Surgery (MIS) Guidelines.
Differential immune infiltrates in histomorphologic Wilms tumor regions identify prognostic macrophages.
📚 EuropePMC975 artigos no totalmostrando 196
International Society of Paediatric Surgical Oncology (IPSO) Minimally Invasive Surgery (MIS) Guidelines.
EcancermedicalscienceDifferential immune infiltrates in histomorphologic Wilms tumor regions identify prognostic macrophages.
Molecular therapy. OncologyUltrasound-Responsive Folate-Chlorogenic Acid Nanocarrier Targeting Mixed Lineage Kinase 1 for Enhanced Wilms Tumor Therapy.
Cancer biotherapy & radiopharmaceuticalsUnderstanding the Burden of Care for Children With Pediatric Solid Tumors.
Pediatric blood & cancerFlavokawain C suppresses nephroblastoma growth by inducing autophagy-mediated downregulation of FABP4 via AMPK/mTOR pathway.
Scientific reportsEXPRESS: ULTRASONOGRAPHIC FEATURES OF FELINE RENAL NEOPLASIA: A RETROSPECTIVE STUDY ON 187 CASES.
Journal of feline medicine and surgeryPredictors of Clinical Outcome in Primary Progressive and Relapsed Wilms Tumor in a Resource-Limited Setting.
CureusAdult Wilms Tumour: A Case Report of an Atypical Presentation and Literature Review.
CureusWilms tumor in adults-an unusual encounter: a case report.
Journal of medical case reportsSomatic Tumor-Like Neoplasms Arising With Testicular Germ Cell Tumors: Distilling Evolving Understanding, Terminology, and Implications for the Practicing Surgical Pathologist.
Advances in anatomic pathologyPrint, plan, perform: Evaluating the clinical impact of anatomical modelling in the surgical treatment of pediatric thoracic and abdominal tumours.
Journal of pediatric surgeryAdult Wilms tumor in an eighty-year-old man: A case report and literature review of recent elderly-onset cases.
International cancer conference journalSerum Levels of CCN3 Protein in Iranian Women with Polycystic Ovary Syndrome.
Journal of reproduction & infertilityMinimally invasive surgery in paediatric nephroblastoma.
Ecancermedicalscience[Retracted] Aberrant expression and mechanism of miR-130b-3p/phosphatase and tensin homolog in nephroblastoma in children.
Experimental and therapeutic medicinePotency of real-time virtual sonography for the preoperative evaluation of invasion in nephroblastoma.
Nagoya journal of medical scienceCognitive and Psychosocial Outcome in Children and Adolescents Treated for Extracranial Malignant Solid Tumors and Lymphomas in the RISK-N Study.
Pediatric blood & cancerLipid metabolism abnormalities in pediatric abdominal solid malignant tumors: a comprehensive review.
Frontiers in pediatricsRelapsed rhabdoid tumours and other non-nephroblastoma childhood and adolescent kidney tumours: perspectives from the HARMONICA collaboration.
Nature reviews. UrologyWilms Tumor Management: A Systematic Review Comparing SIOP and NWTS/COG Protocols in Diagnosis, Treatment, and Outcome.
Asian Pacific journal of cancer prevention : APJCP[Teratoid Wilms tumor in an adult: A case report].
Revista medica del Instituto Mexicano del Seguro SocialUnusual Presentation of Nephroblastoma in an Adolescent: A Case of Initial Misdiagnosis as a Renal Abscess.
CureusPaediatric nephroblastoma at a South African tertiary hospital: A 21-year retrospective analysis.
South African medical journal = Suid-Afrikaanse tydskrif vir geneeskundeBlastemal Tumours: Cytological Features and the Usefulness of Fine Needle Aspiration.
Acta cytologicaMultilocular cystic nephroma in a 15-month-old infant: Successful surgical management and 10-year follow-up.
International journal of surgery case reportsA rare coincidence of intrarenal Wilms tumor with spina bifida: a case report and review of the literature.
International journal of surgery case reportsAdvanced Wilms Tumor in a Middle-Aged Adult: A Case Report.
Clinical case reports[High-throughput circular RNA sequencing reveals tumor-specific high expression of hsa_circ_0001900 in Wilms tumor in association with poor prognosis].
Nan fang yi ke da xue xue bao = Journal of Southern Medical UniversityThe Advancing Frontier: Robotic-Assisted Laparoscopy in Pediatric Tumor Management.
Indian journal of surgical oncologyDouble Trouble-A Case Report of a Rare Second Malignancy in a Neuroblastoma Survivor.
International journal of surgical pathologyThe EMazing Race: A Novel Gamified Board and Clinical Practice Review for Emergency Medicine Residents.
Journal of education & teaching in emergency medicineCase report: Abscopal response and reversal of PD-1 resistance in a patient with nephroblastoma following radiofrequency ablation.
Frontiers in oncologySynthetic organochlorines exert bone anabolic activity by modulating CCN3 signaling in osteoblasts.
Environment internationalCCN3/IMP3/HIF1α positive feedback loop enhances malignant progression of triple-negative breast cancer.
Cellular signallingThe Impact of Lymph Node Ratio for Children with Wilms Tumors: A National Cancer Database Analysis.
CancersCritical Requirement of Senescence-Associated CCN3 Expression in CD44-Positive Stem Cells for Osteoarthritis Progression.
International journal of molecular sciencesClaudin 6 is a suitable target for CAR T-cell therapy in atypical teratoid/rhabdoid brain tumors and other pediatric solid tumors.
Journal for immunotherapy of cancerClassification of small blue round cell tumors by integrating peptide and N-glycan mass imaging spectrometric profiles.
Analytica chimica actaEfficacy of Preoperative Chemotherapy in Patients With Nephroblastoma and Imaging Findings Suggestive of Preoperative Tumor Rupture.
Pediatric blood & cancerA case of nephroblastoma with inferior vena cava invasion arising in a patient with Smith-Magenis syndrome.
Pediatrics international : official journal of the Japan Pediatric SocietyRare Comorbidity of Sickle Cell Disease and Wilms' Tumor: A Case Report.
Case reports in medicineWilms Tumor With Hepatic and Pulmonary Metastases in a Toddler: A Case Report.
CureusA Backwards Approach to GD2 Immunofluorescence in Human Neuroblastoma Tissue Samples: From Staining to Slicing.
CellsRetroperitoneal extrarenal Wilms tumor: A diagnostic challenge and kidney-sparing approach.
Radiology case reportsCystic wilms tumor, cystic partially differentiated nephroblastoma, or cystic nephroma? A case report and review of the literature.
Urology case reports[Therapeutic adaptations: Rationale, achievements and limits. Experience of GFAOP].
Bulletin du cancerCase Report: Extra-adrenal retroperitoneal paraganglioma in a young adult cat diagnosed by imaging, pathology, and immunohistochemistry.
Frontiers in veterinary sciencePredictors of Surgical Complications and Survival in Pediatric Wilms' Tumor: A 20-Year Retrospective Study from Two Thai Centers.
Current oncology (Toronto, Ont.)Prenatal diagnosis and postnatal management of perinatal thoracoabdominopelvic tumors: multicenter experience.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyAnthracycline-Induced Cardiomyopathy After Nephro-/Neuroblastoma in Childhood: The Importance of Cardiological Reference Assessment.
Cancer medicineTesticular teratoma with nephroblastoma subtype of somatic type malignancy.
Medical journal, Armed Forces IndiaCurrent clinical and surgical insights in the workup and management of Wilms tumor (nephroblastoma).
Current opinion in pediatricsDual PI3K/mTOR inhibitor NVP-BEZ235 induces cell cycle arrest via autophagy mediated protein degradation of RPL19 in nephroblastoma cell.
Frontiers in pharmacologyPulmonary cysts as a diagnostic indicator of Birt-Hogg-Dubé syndrome in patients with renal neoplasm.
Insights into imagingWilms Tumor, Version 2.2025, NCCN Clinical Practice Guidelines In Oncology.
Journal of the National Comprehensive Cancer Network : JNCCNEpigenetic Regulation in Wilms Tumor.
BiomedicinesTargeted therapy for Wilms tumor: a bibliometric study of worldwide scientific activity and collaborative networks.
Discover oncologyAnti-Tumor Activity of Paclitaxel-Containing Regimens in Recurrent/Refractory Wilms Tumor.
Pediatric blood & cancerClinical Indication-Based Pediatric Diagnostic Reference Level Values for Abdominal Computed Tomography: A Descriptive Cross-Sectional Study.
Health science reportsSurgical and diagnostic management challenges of giant Wilms tumor with intracardial and IVC thrombus extension in a 3-year-old: A rare case from a resource-limited facility involving global and local pediatric surgical teams.
International journal of surgery case reportsCCN5 negatively regulates TGF-β-induced endometriosis associated fibrosis through Wnt/β-catenin signaling via Smad3-dependent mechanism.
Journal of translational medicinePatterns of Care and Survival of Wilms Tumor in Children in India: A Retrospective Multicentric INPHOG Study.
Pediatric blood & cancerManagement of Anaesthesia and Cardiopulmonary Bypass in Paediatric Patients With Abdominal Tumours Invading the Inferior Vena Cava and Right Atrium: A Case Series of a Tertiary Children's Medical Centre in China.
Cancer reports (Hoboken, N.J.)Prognostic and Therapeutic Value of Metabolism-Related Genes in Nephroblastoma: A Focus on the Key Gene NNMT and Its Regulative Effect on Metabolism.
Cell biochemistry and functionPaediatric renal tumors: An insight into molecular characteristics, histomorphology and syndromic association.
World journal of nephrologyChildhood Cancer in Zimbabwe: A Review of KidzCan's Database.
JCO global oncologyWilms tumor characteristics in children with heterozygous germline DIS3L2 variants.
Genetics in medicine : official journal of the American College of Medical GeneticsMulti-Observer Study on Diagnostic Accuracy of Pediatric Renal Tumors Imaged with Higher-Harmonic-Generation Microscopy.
CancersDynamic changes in Ccn3 expression across the limbic forebrain through the mouse estrous cycle and during lactation.
Journal of neuroendocrinologyExtrarenal Wilms tumor in the retroperitoneum of a 6-year-old girl: a case report and review of the literature.
Journal of medical case reportsDistinct pathways for genetic and epigenetic predisposition in familial and bilateral Wilms tumor.
Genome medicineComputed tomography based radiomics signature for predicting the expression of vascular endothelial growth factor in pediatric patients with nephroblastoma.
Scientific reportsExploring the in silico adaptation of the Nephroblastoma Oncosimulator to MRI scans, treatment data, and histological profiles of patients from different risk groups.
Frontiers in physiologySpatial and Temporal Distribution of Pediatric Cancers in Southern Ghana: A Retrospective Observational Study.
Health science reportsAt the crossroad between Ewing sarcoma and neuroblastoma: a report of two cases of Ewing sarcoma with post treatment neuroblastoma-like differentiation.
Diagnostic pathologyThe impact of pre-operative angiography on nephron-sparing surgery and outcome in wilms tumor arising from horseshoe kidney: case report and literature review.
Frontiers in surgeryA rare case of concurrent Wilms' tumor in the left kidney and left ovary.
Urology case reportsMetastatic canine intraspinal nephroblastoma following spinal cord decompression in a Golden Retriever.
Journal of the American Veterinary Medical AssociationNephroblastoma in a Little Skate, Leucoraja erinaceus (Mitchill, 1825).
Journal of fish diseasesThe signature based on interleukin family and receptors identified IL19 and IL20RA in promoting nephroblastoma progression through STAT3 pathway.
Scientific reportsBlastemal predominant WT1 negative Wilms tumour of the young adult: a unique case report and review of the literature.
Frontiers in medicineNephroblastoma Overexpressed Protein (NOV/CCN3) Elevated Expression of Inflammation Regulators in a Model of Sepsis-Induced Lung Injury.
Bulletin of experimental biology and medicineRenal Autotransplantation for Resection of Bilateral Nephroblastoma and High-Risk Neuroblastoma in Children.
CancersWilms' Tumor: A Review of Clinical Characteristics, Treatment Advances, and Research Opportunities.
Medicina (Kaunas, Lithuania)Prescription patterns of supportive care medications among children receiving chemotherapy treatments at a major referral hospital in Tanzania: where are we in managing chemotherapy-induced toxicities?
Frontiers in oncologySecondary ovarian germ cell tumor following Wilms tumor after eight years post-treatment: A rare case report.
International journal of surgery case reportsExpression and clinical significance of CCN5 and the oestrogen receptor in advanced breast cancer.
BMC women's healthPrognostic Correlation of Basic Fibroblast Growth Factor and Vascular Endothelial Growth Factor with Radiological Tumor Size in Pediatric Nephroblastoma and Neuroblastoma: A Prospective Study.
Journal of Indian Association of Pediatric SurgeonsA Neonate with Unilateral Nonsyndromic Wilms' Tumor Treated with Nephron-sparing Surgery: The Youngest Ever Reported in the World.
Journal of Indian Association of Pediatric SurgeonsUtilization of DNA Replication factor MCM2 by Cancer.
Current cancer drug targetsPaediatric Renal Tumors: A State-of-the-Art Review.
Current oncology reportsA rare case of nephroblastoma arising in a multicystic dysplastic kidney: a case report and review of the literature.
Journal of surgical case reportsMetanephric stromal tumor as a rare differential diagnosis of a renal mass in children - a case report.
Pediatric radiologyAdult Wilms Tumor With Inferior Vena Cava Thrombus on an Incomplete Duplex Collecting System Ureter Fissus Proximalis Managed at a Tertiary Hospital in Tanzania: A Case Report and Literature Review.
Clinical case reportsA Highly Bioactive Organic-Inorganic Nanoparticle for Activating Wnt10b Mediated Osteogenesis by Specifically Anchor CCN3 Protein.
Advanced healthcare materialsThe high levels of fibrinogen and platelets are associated with poor survival in nephroblastoma in children.
Translational cancer researchPaediatric renal tumours: an update on challenges and recent developments.
Virchows Archiv : an international journal of pathologyImpact of Hospital Volume on Survival for Pediatric Wilms Tumor.
Pediatric blood & cancerMiR-196a2 rs11614913 C Allele is Associated with Increased Wilms Tumor Susceptibility in Chinese Children.
Journal of CancerWilms tumor primary cultures capture phenotypic heterogeneity and facilitate preclinical screening.
Translational oncologyIntegrated analyses of Mendelian randomization, eQTL, and single-cell transcriptome identify CCN3 as a potential biomarker in aortic dissection.
Scientific reportsBilateral Wilms Tumor - Case Report of a Patient with Family History.
The application of clinical geneticsExploring the Role of Ccn3 in Type III Cell of Mice Taste Buds.
Journal of neurochemistryCancer Stage Geospatial Analysis to Optimize Educational Activities in Pediatric Oncology and Hematology: A Retrospective Cohort Study.
Pediatric blood & cancerThree novel neoplasms in Nancy Ma's owl monkeys (Aotus nancymaae).
Veterinary pathologyRare cases of a second recurrence of nephroblastoma with MLLT1 gene mutation: case report and literature review.
Frontiers in oncologyWilms tumor and associated predisposing syndromes and conditions.
JAAPA : official journal of the American Academy of Physician AssistantsA Unique Case of Cystic Partially Differentiated Nephroblastoma Associated With Botryoid Intralobar Nephrogenic Rests.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyMediastinal Teratoma with Nephroblastomatous Elements: Case Report, Literature Review, and Comparison with Maturing Fetal Glomerulogenic Zone/Definitive Zone Ratio and Nephrogenic Rests.
International journal of molecular sciencesAcute pyelonephritis and subcapsular hematoma revealing a nephroblastoma.
Radiology case reportsCellular communication network factor 3 contributes to the pathological process of rheumatoid arthritis through promoting cell senescence and osteoclastogenesis in the joint.
Journal of autoimmunityCCN3/NOV as a potential therapeutic target for diverticular disease: A proteome-wide Mendelian randomization study.
MedicineAtypical presentation of Wilms' tumor in an adult: a case report and diagnostic considerations.
Journal of surgical case reportsLong-Term Survey of Japanese Children with Recurrent Nephroblastoma: A Report from Japan Children's Cancer Group.
Pediatric hematology and oncologyA review on optimization of Wilms tumour management using radiomics.
BJR openZSTK474 targeting PIK3R3 inhibits the Wilms' tumor through G0 / G1 phase arrest.
PloS oneGenetic analysis using next-generation sequencing and multiplex ligation probe amplification in Chinese aniridia patients.
Orphanet journal of rare diseasesAdult nephroblastoma or Wilms' tumor: A rare entity - Case report.
Urology case reportsReflections on 20 years of the Wilms Africa project: Lessons learned and the way forward.
Pediatric blood & cancerFeatures of Metabolites and Biomarkers in Inflammatory and Infectious Complications of Childhood Cancers.
BiomedicinesSupernumerary kidney fused to the isthmus of a horseshoe kidney, with subsequent nephroblastoma, identified with dynamic renal scintigraphy.
Revista espanola de medicina nuclear e imagen molecularCase report: Metastatic BRAF V600E-mutated adult Wilms' tumor with robust response to BRAF/MEK inhibitor therapy.
Frontiers in oncologyLaparoscopic Treatment of Wilms' Tumor: Criteria of SIOP-UMBRELLA Protocol may be Updated.
Annals of surgical oncologyWilms Tumor with Vena Caval Intravascular Extension: A Surgical Perspective.
Children (Basel, Switzerland)Effectiveness of a Wilms tumour treatment guideline adapted to local circumstances in sub-Saharan Africa: A report from Wilms Africa Phase II-CANCaRe Africa.
Pediatric blood & cancerFactors Associated with Outcomes at 1 Year in Paediatric Post-nephrectomy Patients for Nephroblastoma at the University Teaching Hospital and Cancer Diseases Hospital in Lusaka, Zambia.
African journal of paediatric surgery : AJPSWTAP and METTL14 regulate the m6A modification of DKK3 in renal tubular epithelial cells of diabetic nephropathy.
Biochemical and biophysical research communicationsNephroblastoma in Older Adult: Case Report and Review of Literature.
Case reports in oncologyAmino-truncated NOV expression and its correlation with clinicopathologic features, prognosis, metastasis, and chemoresistance in bladder cancer.
Cancer biology & therapyConcomitant Wilms tumor and autosomal dominant polycystic kidney disease.
Pediatric blood & cancerCharacteristics and outcome of synchronous bilateral Wilms tumour in the SIOP WT 2001 Study: Report from the SIOP Renal Tumour Study Group (SIOP-RTSG).
British journal of cancerInsights into Personalized Care Strategies for Wilms Tumor: A Narrative Literature Review.
BiomedicinesCCN3 maintains bone density during lactation.
Nature reviews. Endocrinology[Laparoscopic nephrectomies and kidney resections in children].
KhirurgiiaSynovial Sarcoma of the Kidney: Diagnostic Pitfalls in a Case with Myxoid Monophasic Differentiation and No Epithelial Biomarkers Expression.
International journal of molecular sciencesA maternal brain hormone that builds bone.
NatureThe Pattern of Anemia in Pediatric Solid Tumors Prior to and after Chemotherapy- A Retrospective Cohort Study.
Current cancer drug targetsSurgical aspects and outcomes after nephrectomy for Wilms tumour in sub-Saharan Africa: A report from Wilms Africa Phase II-CANCaRe Africa.
Pediatric blood & cancerFirst incidence of extrarenal wilms tumor within the spinal canal in the adult population: a novel case report and literature review.
BMC urologyA Multidisciplinary Hyper-Modeling Scheme in Personalized In Silico Oncology: Coupling Cell Kinetics with Metabolism, Signaling Networks, and Biomechanics as Plug-In Component Models of a Cancer Digital Twin.
Journal of personalized medicineThe impact of the route to diagnosis in nephroblastoma.
Cancer medicineClinical Characteristics and Prognoses of Mucormycosis in Four Children.
Infection and drug resistancePrevention of treatment abandonment remains an important challenge to increase survival of Wilms tumor in sub-Saharan Africa: A report from Wilms Africa-CANCaRe Africa.
Pediatric blood & cancerEarly myocardial injury in children on doxorubicin for cancer chemotherapy: a cross-sectional study in a tertiary referral centre in Kenya.
BMC cardiovascular disordersA population-based study on incidence trends of kidney and renal pelvis cancers in the United States over 2000-2020.
Scientific reports[Identification of key genes in Wilms tumor based on high-throughput RNA sequencing and their impacts on prognosis and immune responses].
Nan fang yi ke da xue xue bao = Journal of Southern Medical UniversityUsing imaging to diagnose renal tumors beyond nephroblastoma.
Radiology case reportsSpinal metastasis of nephroblastoma: Yes it exists.
Radiology case reportsSurgical Management of Wilms Tumors with Intravenous Extension: A Multicenter Analysis of Clinical Management with Technical Insights.
Annals of surgical oncologyCase Report: Autosomal dominant polycystic kidney disease and Wilms' tumor in infancy and childhood.
Frontiers in pediatricsCancer patterns in Arua district, Uganda: a hospital-based retrospective study.
EcancermedicalscienceNephroblastoma-specific dysregulated gene SNHG15 with prognostic significance: scRNA-Seq with bulk RNA-Seq data and experimental validation.
Discover oncologyHypoxia/inflammation-induced upregulation of HIF-1α and C/EBPβ promotes nephroblastoma cell EMT by improving HOXA11-AS transcription.
HeliyonCorrelation of CT Scan Response to Necrosis and Volume Changes With Histopathology in Children With Wilms' Tumor.
Journal of pediatric surgeryPrimary testicular teratoid Wilms tumor in a 40-year-old male with retroperitoneal lymph node involvement: A case report.
Urology case reportsCongenital Tooth Agenesis and Risk of Early-Onset Cancer.
JAMA network openBilateral Wilms tumor: 10-year experience from a single center in China.
Translational cancer researchmiRNAs Role in Wilms tumor pathogenesis: Signaling pathways interplay.
Pathology, research and practiceMulticystic renal dysplasia, a histomorphological spectrum: Seven years experience from a tertiary care hospital.
Indian journal of pathology & microbiologyCCN3/NOV Serum Levels in Non-alcoholic Fatty Liver Disease (NAFLD) Patients in Comparison with the Healthy Group and its Correlation with TNF-α and IL-6.
Current molecular medicineSuspected renal interstitial cell tumor causing polycythemia in two dogs.
The Journal of veterinary medical scienceMechanism of LncRNA FTX regulates nephroblastoma progression through MiR-215-5p/PI3K/AKT axis.
Journal of pediatric urologyOverexpression of aquaporin-1 plays a vital role in proliferation, apoptosis, and pyroptosis of Wilms' tumor cells.
Journal of cancer research and clinical oncologyA Comprehensive Study on the Association between Plasma NOV/CCN3 Levels and Insulin Resistance in Childhood Obesity.
Annals of nutrition & metabolismWilms tumour resulting from paternal transmission of a TRIM28 pathogenic variant-A first report.
European journal of human genetics : EJHGBilateral Nephroblastomatosis With a Unilateral Wilms Tumor: A Case Report Highlighting Imaging Characteristics.
Global pediatric healthSingle-cell transcriptomes of kidneys in a 6-month-old boy with Denys-Drash syndrome reveal stromal cell heterogeneity in the tumor microenvironment.
Clinical kidney journalCCN3/NOV inhibition attenuates oxidative stress-induced apoptosis of mouse neural stem/progenitor cells by blocking the activation of p38 MAPK: An in vitro study.
Brain researchMultidimensional Transcriptomics Unveils RNF34 as a Prognostic Biomarker and Potential Indicator of Chemotherapy Sensitivity in Wilms' Tumour.
Molecular biotechnologyCharacteristics and Clinical Outcomes of Children With Wilms' Tumour: A 15-year Experience in a Single Centre in Nigeria.
Journal of pediatric surgeryA rare case of extrarenal Wilms tumor of the uterine corpus: comprehensive genomic profile and review of the literature.
PathologicaRobotic-assisted laparoscopic surgery for the treatment of Wilms' tumor in children: single-center experience and medium-term outcomes.
Journal of robotic surgeryAltered Red Blood Cell Fatty Acid and Serum Adipokine Profiles in Subjects with Obesity.
BiomedicinesCystic partially differentiated nephroblastoma in an 18-month-old girl: a case report.
Annals of medicine and surgery (2012)Teratoma-associated and so-called pure Wilms tumour of the ovary represent two separate tumour types with distinct molecular features.
HistopathologyComplex Chromosomal Rearrangement Involving Chromosomes 10 and 11, Accompanied by Two Adjacent 11p14.1p13 and 11p13p12 Deletions, Identified in a Patient with WAGR Syndrome.
International journal of molecular sciencesRedo nephron-sparing surgery in stage V pediatric renal tumors - A report from the SIOP/GPOH study group for renal tumors.
European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical OncologyWilms Tumor in India: A Systematic Review.
South Asian journal of cancerRisks of long-term mortality and chronic health conditions experienced by Wilms tumor survivors.
Translational pediatricsS100A16 cooperates with DEPDC1 to promote the progression and angiogenesis of nephroblastoma through PI3K/Akt/mTOR pathway.
Polish journal of pathology : official journal of the Polish Society of PathologistsRenal cell carcinoma in the contralateral kidney with TFE3 gene translocation following chemotherapy for childhood nephroblastoma: A case report and literature review.
Clinical case reports[Renal tumors in children and adolescents].
Radiologie (Heidelberg, Germany)Identification of m6A-associated genes as prognostic and immune-associated biomarkers in Wilms tumor.
Discover oncologyManagement of Macroglossia Secondary to Beckwith-Weidmann Syndrome in a Pediatric Patient: A Case Report.
CureusWilms tumor in horseshoe kidney with extensive vascular thrombosis: A case report.
International journal of surgery case reportsTraumatic rupture of nephroblastoma misdiagnosed as isolated renal rupture in children.
Asian journal of surgeryTRIM28 inactivation in epithelial nephroblastoma is frequent and often associated with predisposing TRIM28 germline variants.
The Journal of pathologySimultaneous Occurrence of Multiple Neoplasms in Children with Cancer Predisposition Syndromes: Collaborating with Abnormal Genes.
GenesDisseminated adult Wilms tumor in pregnancy: Leveraging multidisciplinary care in a low-resource setting.
International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and ObstetricsChanging pattern of cancer distribution: experience from a tertiary health institution in Nigeria and review of literature.
Nigerian journal of clinical practiceUnwrapping Nephrogenic Rests and Nephroblastomatosis for Pediatric Surgeons: A Systematic Review Utilizing the PICO Model by the APSA Cancer Committee.
Journal of pediatric surgeryMosaic TP53 Mutation on Tumour Development in Pigs: A Case Study.
Veterinary medicine internationalThe rationale for nephron-sparing surgery in unilateral non-syndromic Wilms tumour.
Pediatric nephrology (Berlin, Germany)Recent Improvements in Adult Wilms Tumor Diagnosis and Management: Review of Literature.
Journal of kidney cancer and VHLA novel model incorporating chromatin regulatory factors for risk stratification, prognosis prediction, and characterization of the microenvironment in Wilms tumor.
The journal of gene medicineA spectrum of findings on computed tomography in paediatric abdominal and pelvic tumours in a Ghanaian teaching hospital.
Ghana medical journalAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- International Society of Paediatric Surgical Oncology (IPSO) Minimally Invasive Surgery (MIS) Guidelines.
- Differential immune infiltrates in histomorphologic Wilms tumor regions identify prognostic macrophages.
- Understanding the Burden of Care for Children With Pediatric Solid Tumors.
- Cognitive and Psychosocial Outcome in Children and Adolescents Treated for Extracranial Malignant Solid Tumors and Lymphomas in the RISK-N Study.
- Relapsed rhabdoid tumours and other non-nephroblastoma childhood and adolescent kidney tumours: perspectives from the HARMONICA collaboration.
- Genome-Wide Copy-Number Landscape of Germ Cell Tumors With Synchronous Conventional and "Somatic-Type" Malignancy Components.
- [Wilms' tumor in a horseshoe kidney: organ-sparing treatment].
- [Paediatric kidney tumours: Update on the challenges of nephroblastoma and other kidney tumours].
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:654(Orphanet)
- MONDO:0019004(MONDO)
- GARD:7892(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
