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Wilms' tumor

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276: 53: 291:(WT1) or Wilms Tumor 2 (WT2) genes, and the tumor presents with a group of other signs and symptoms. Non-syndromic Wilms' tumor is not associated with other symptoms or pathologies. Many, but not all, cases of Wilms' tumor develop from nephrogenic rests, which are fragments of tissue in or around the kidney that develop before birth and become cancerous after birth. In particular, cases of bilateral Wilms' tumor, as well as cases of Wilms' tumor derived from certain genetic syndromes such as 268: 295:, are strongly associated with nephrogenic rests. Most nephroblastomas are on one side of the body only and are found on both sides in less than 5% of cases, although people with Denys-Drash syndrome mostly have bilateral or multiple tumors. They tend to be encapsulated and vascularized tumors that do not cross the midline of the abdomen. In cases of 1332:
van den Heuvel-Eibrink, Marry M.; Hol, Janna A.; Pritchard-Jones, Kathy; van Tinteren, Harm; Furtwängler, Rhoikos; Verschuur, Arnauld C.; Vujanic, Gordan M.; Leuschner, Ivo; Brok, Jesper; Rübe, Christian; Smets, Anne M.; Janssens, Geert O.; Godzinski, Jan; Ramírez-Villar, Gema L.; de Camargo, Beatriz
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Tumor-specific loss-of-heterozygosity (LOH) for chromosomes 1p and 16q identifies a subset of Wilms' tumor patients who have a significantly increased risk of relapse and death. LOH for these chromosomal regions can now be used as an independent prognostic factor together with disease stage to target
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Messahel B, Williams R, Ridolfi A, A'hern R, Warren W, Tinworth L, Hobson R, Al-Saadi R, Whyman G, Brundler MA, Kelsey A, Sebire N, Jones C, Vujanic G, Pritchard-Jones K (March 2009). "Allele loss at 16q defines poorer prognosis Wilms tumour irrespective of treatment approach in the UKW1-3 clinical
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5% of Wilms' tumor cases at the time of initial diagnosis are bilateral involvements, which pose unique challenges to treatment. An attempt should be made to stage each side according to the above criteria (stage I to III) on the basis of extent of disease prior to biopsy. Bilateral Wilms' tumors
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Coorens THH, Treger TD, Al-Saadi R, Moore L, Tran MGB, Mitchell TJ, Tugnait S, Thevanesan C, Young MD, Oliver TRW, Oostveen M, Collord G, Tarpey PS, Cagan A, Hooks Y, Brougham M, Reynolds BC, Barone G, Anderson J, Jorgensen M, Burke GAA, Visser J, Nicholson JC, Smeulders N, Mushtaq I, Stewart GD,
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Wilms' tumor affects approximately one person per 10,000 worldwide before the age of 15 years. People of African descent may have slightly higher rates of Wilms' tumor. The peak age of Wilms' tumor is 3 to 4 years and most cases occur before the age of 10 years. A genetic predisposition to Wilms'
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The majority of people with Wilms' tumor present with an asymptomatic abdominal mass which is noticed by a family member or healthcare professional. Renal tumors can also be found during routine screening in children who have known predisposing clinical syndromes. The diagnostic process includes
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Approximately 650 cases are diagnosed in the U.S. annually. The majority of cases occur in children with no associated genetic syndromes; however, a minority of children with Wilms' tumor have a congenital abnormality.  It is highly responsive to treatment, with about 90 percent of children
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Wilms tumor is the most common malignant renal tumor in children. There are a number of rare genetic syndromes that have been linked to an increased risk of developing Wilms Tumor. Screening guidelines vary between countries; however health care professionals are recommending regular ultrasound
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Tournade MF, Com-Nougué C, de Kraker J, Ludwig R, Rey A, Burgers JM, Sandstedt B, Godzinski J, Carli M, Potter R, Zucker JM (January 2001). "Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms' tumor in children older than 6 months: results of the Ninth International
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Fitski, Matthijs; van de Ven, Cornelis P.; Hulsker, Caroline C. C.; Bökkerink, Guus M. J.; Terwisscha van Scheltinga, Cecilia E. J.; van den Heuvel-Eibrink, Marry M.; Mavinkurve-Groothuis, Annelies M. C.; van Grotel, Martine; Wijnen, Marc H. W. A.; Klijn, Aart J.; van der Steeg, Alida F. W.
1120:, founder of Dana–Farber Cancer Institute, and his colleagues achieved the first remissions in Wilms' tumor in the 1950s. By employing the antibiotic actinomycin D in addition to surgery and radiation therapy, they boosted cure rates from 40 to 89 percent. 1754:
Campbell PJ, Wedge DC, Martincorena I, Rampling D, Hook L, Warren AY, Coleman N, Chowdhury T, Sebire N, Drost J, Saeb-Parsy K, Stratton MR, Straathof K, Pritchard-Jones K, Behjati S (2019) Embryonal precursors of Wilms tumor. Science 366(6470):1247-1251
545: 1131:, an Italian physician. He discovered that in the direct radiograms and in the urographic images, you can identify determining elements for a differential diagnosis with the Wilms' tumor. This possibility was a premise for starting a treatment. 2487: 2472: 1541:
Call KM, Glaser T, Ito CY, Buckler AJ, Pelletier J, Haber DA, Rose EA, Kral A, Yeger H, Lewis WH (February 1990). "Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locus".
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Stewénius Y, Jin Y, Øra I, de Kraker J, Bras J, Frigyesi A, Alumets J, Sandstedt B, Meeker AK, Gisselsson D (November 2007). "Defective chromosome segregation and telomere dysfunction in aggressive Wilms' tumors".
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Pritchard-Jones K, Fleming S, Davidson D, Bickmore W, Porteous D, Gosden C, Bard J, Buckler A, Pelletier J, Housman D (July 1990). "The candidate Wilms' tumour gene is involved in genitourinary development".
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Cut section showing two halves of a nephroblastoma specimen. Note the prominent septa subdividing the sectioned surface and the protrusion of tumor into the renal pelvis, resembling botryoid rhabdomyosarcoma.
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Spreafico F, Pritchard Jones K, Malogolowkin MH, Bergeron C, Hale J, de Kraker J, Dallorso S, Acha T, de Camargo B, Dome JS, Graf N (December 2009). "Treatment of relapsed Wilms tumors: lessons learned".
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Guaragna MS, Soardi FC, Assumpção JG, Zambaldi L, Cardinalli IA, Yunes JA, de Mello MP, Brandalise SR, Aguiar S (August 2010). "The novel WT1 gene mutation p.H377N associated to Denys-Drash syndrome".
2080:, Li S, Perlman E, Beckwith JB, Ritchey ML, Shamberger RC, Haase GM, D'Angio GJ, Donaldson M, Coppes MJ, Malogolowkin M, Shearer P, Thomas PR, Macklis R, Tomlinson G, Huff V, Green DM (October 2005). 279:
Low magnification micrograph of a Wilms' tumor infiltrating the renal parenchyma. It shows the characteristic triphasic pattern consisting of tubules, solid sheets of small round cells, and stroma.
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findings and tumor cells pathology. According to the extent of tumor tissue at the time of initial diagnosis, four stages are considered, with a fifth classification for bilateral involvement.
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Kalish JM, Doros L, Helman LJ, Hennekam RC, Kuiper RP, Maas SM, Maher ER, Nichols KE, Plon SE, Porter CC, Rednam S, Schultz KA, States LJ, Tomlinson GE, Zelley K, Druley TE (July 2017).
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Stage IV (10% of cases) Wilms' tumor is defined by the presence of hematogenous metastases (lung, liver, bone, or brain), or lymph node metastases outside the abdominopelvic region.
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Wilms' tumor has many causes, which can broadly be categorized as syndromic and non-syndromic. Syndromic causes of Wilms' tumor occur as a result of alterations to genes such as the
2502: 3455: 331:, stromal and epithelial derivatives. Characteristic is the presence of abortive tubules and glomeruli surrounded by a spindled cell stroma. The stroma may include striated 1400:
Erginel B, Vural S, Akın M, Karadağ CA, Sever N, Yıldız A. et al (2014) Wilms' tumor: a 24-year retrospective study from a single center. Pediatr Hematol Oncol 31: 409–414
2082:"Loss of heterozygosity for chromosomes 1p and 16q is an adverse prognostic factor in favorable-histology Wilms tumor: a report from the National Wilms Tumor Study Group" 731:
Statistics may sometimes show more favorable outcomes for more aggressive stages than for less aggressive stages, which may be caused by more aggressive treatment and/or
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and peritoneal dissemination of the tumor. In such cases, surgical intervention by a surgeon who is experienced in the removal of such a fragile tumor is imperative.
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Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling followed by abdominal radiotherapy, whole-lung radiotherapy
3571: 1285:"Patient-specific hydrogel phantoms for the preoperative simulation of nephron-sparing surgery in Wilms' tumor patients: A feasibility study" 1086:, followed by nephron sparing surgery or nephrecomy, staging of tumors, and chemotherapy and/or radiotherapy based on pathology and staging 1068:, followed by nephron sparing surgery or nephrecomy, staging of tumors, and chemotherapy and/or radiotherapy based on pathology and staging 1050:, followed by nephron sparing surgery or nephrecomy, staging of tumors, and chemotherapy and/or radiotherapy based on pathology and staging 3256: 3103: 2869: 593:
Staging is a standard way to describe the extent of spread of Wilms' tumors and to determine prognosis and treatments. Staging is based on
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in the study groups. Also, a stage V tumor is not necessarily worse than, but nevertheless comparable in prognosis to a stage IV tumor.
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can also be used for more detailed imaging. Finally, the diagnosis of Wilms' tumor is confirmed by a tissue sample. In most cases, a
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Dome JS, Graf N, Geller JI, Fernandez CV, Mullen EA, Spreafico F, Van den Heuvel-Eibrink M, Pritchard-Jones K (September 2015).
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Characterized by kidney diseases since birth leading to early-onset kidney failure, ambiguous genitalia (intersex disorders).
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In case of relapse of Wilms' tumor, the 4-year survival rate for children with a standard-risk has been estimated to be 80%.
1262: 2249:"Surveillance Recommendations for Children with Overgrowth Syndromes and Predisposition to Wilms Tumors and Hepatoblastoma" 988:
Nephrectomy + lymph node sampling, followed by abdominal radiotherapy, bilateral pulmonary radiotherapy, and regimen DD-4A
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Nephrectomy + lymph node sampling, followed by abdominal radiotherapy, bilateral pulmonary radiotherapy, and regimen DD-4A
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Once Wilms' tumor is suspected, an ultrasound scan is usually done first to confirm the presence of an intrarenal mass. A
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is not done first because there is a risk of cancer cells spreading during the procedure. Treatment in North America is
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Nephroblastoma in childhood: current possibilities for an early radiographic diagnosis, Italian Journal of Surgery 1969
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Immediate nephrectomy + lymph node sampling followed by abdominal radiotherapy, whole-lung radiotherapy, and regimen I
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The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank.
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followed by nephrectomy. A definitive diagnosis is obtained by pathological examination of the nephrectomy specimen.
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Preoperative treatment with regimen DD-4A followed by nephrectomy + lymph node sampling and abdominal radiotherapy
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intensity of treatment to risk of treatment failure. Genome-wide copy number and LOH status can be assessed with
470: 399:. In addition at least half of the Wilms' tumors with mutations in WT1 also carry acquired somatic mutations in 343:, fat tissue, and fibrous tissue. Dysfunction is caused when the tumor compresses the normal kidney parenchyma. 3041: 2527: 1621: 943:
Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling and abdominal radiotherapy
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Tumor spillage involving peritoneal surfaces either before or during surgery, or transected tumor thrombus.
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taking a medical history, a physical exam, and a series of tests including blood, urine, and imaging tests.
2336:"Age distributions, birth weights, nephrogenic rests, and heterogeneity in the pathogenesis of Wilms tumor" 1214: 752: 3576: 3305: 3156: 3113: 3108: 716:
is estimated to be approximately 90%, but for individuals the prognosis is highly dependent on individual
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Wilms' tumors may be separated into two prognostic groups based on pathologic characteristics:
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The mesenchymal component may include cells showing rhabdomyoid differentiation or malignancy (
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Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma.
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Sonn G, Shortliffe LM (October 2008). "Management of Wilms tumor: current standard of care".
1707:"Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration" 1417:"Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Health Professional Version" 957:
Immediate nephrectomy + lymph node sampling followed by abdominal radiotherapy and regimen I
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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Health Professional Version
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for the diagnosis of Wilms' tumor began in the early 1970s, thanks to the intuition of
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Nephrectomy + lymph node sampling followed by abdominal radiotherapy and regimen DD-4A
746: 741: 387:(11p13) are observed in approximately 20% of Wilms' tumors, the majority of them being 2496: 252:
in some cases (especially if synchronous or metachronous bilateral kidney involvement)
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Favorable histology in children younger than 24 months or tumor weight less than 550g
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In Stage I Wilms' tumor (43% of cases), all of the following criteria must be met:
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Favorable histology in children older than 24 months or tumor weight more than 550g
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Breslow N, Olshan A, Beckwith JB, Green DM (1993). "Epidemiology of Wilms tumor".
2264: 1974: 1491: 280: 208:), and occurs most commonly as a renal tumor in child patients. It is named after 65: 2532: 1460: 1389: 898:
Nephrectomy + lymph node sampling followed by abdominal radiotherapy and regimen
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Nephrectomy + lymph node sampling followed by abdominal radiotherapy and regimen
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it is usually to the lung. A rupture of Wilms' tumor puts the patient at risk of
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In Stage III (20% of cases), 1 or more of the following criteria must be met:
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In Stage II (23% of cases), 1 or more of the following criteria must be met:
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10.1002/(SICI)1096-8628(19981002)79:4<260::AID-AJMG6>3.0.CO;2-Q
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Surgery only (should be done only within the context of a clinical trial)
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has been established, due to deletions in the p13 band on chromosome 11.
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Nephrectomy + lymph node sampling followed by abdominal radiotherapy and
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The tumor is not ruptured or biopsied (open or needle) prior to removal.
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Pathologically, a triphasic nephroblastoma comprises three elements:
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Typical signs and symptoms of Wilms' tumor include the following:
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No involvement of extrarenal or renal sinus lymph-vascular spaces
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Breslow NE, Beckwith JB, Perlman EJ, Reeve AE (September 2006).
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No residual tumor apparent at or beyond the margins of excision.
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trials: a Children's Cancer and Leukaemia Group (CCLG) Study".
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Popov, Sergey D.; Sebire, Neil J.; Vujanic, Gordan M. (2016).
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Society of Pediatric Oncology Wilms' Tumor Trial and Study".
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Nephrectomy + lymph node sampling followed by regimen EE-4A
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Mukherjee, Siddhartha, The Emperor of All Maladies, pg. 123
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Tumor extends beyond the kidney but is completely excised.
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No residual tumor apparent beyond the margins of excision.
1215:"Wilms Tumor and Other Childhood Kidney Tumors Treatment" 212:, the German surgeon (1867–1918) who first described it. 87: 2139:
Unless otherwise specified in boxes, then reference is:
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screening for people with associated genetic syndromes.
510:(one side of the body is larger), other tumors in body, 437:(Wilms tumor, aniridia, genital anomalies, retardation) 414:
Most cases do not have mutations in any of these genes.
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Extensive tumor involvement of renal sinus soft tissue.
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Multiple cutaneous and uterine leiomyomatosis syndrome
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GeneReviews/NCBI/NIH/UW entry on Wilms' Tumor Overview
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Nephrectomy + lymph node sampling followed by regimen
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of 11 cm Wilms' tumor of right kidney in 13-month-old.
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Szychot E, Apps J, Pritchard-Jones K (January 2014).
1822:"Presentation, diagnosis, and staging of Wilms tumor" 368:– Contains diffuse anaplasia (poorly developed cells) 96: 2454: 1493:
Wilms' Tumour – Histology and Differential Diagnosis
720:. Early removal tends to promote positive outcomes. 360:– Contains well developed components mentioned above 90: 84: 3531: 3468: 3359: 3335: 3313: 3304: 3226: 3198: 3170: 3126: 3085: 3000: 2973: 2964: 2955: 2929: 2862: 2829: 2801: 2783: 2753: 2710: 2689: 2680: 2564: 2458: 167: 159: 149: 139: 131: 111: 93: 72: 40: 35: 1620:Maiti S, Alam R, Amos CI, Huff V (November 2000). 632:Metastasis of tumor to lymph nodes not identified. 1651:Ruteshouser EC, Robinson SM, Huff V (June 2008). 1840:"Wilms' tumor: biology, diagnosis and treatment" 1767:PDQ Pediatric Treatment Editorial Board (2002). 1415:PDQ Pediatric Treatment Editorial Board (2002), 651:Any of the following conditions may also exist: 1585:Huff V (October 1998). "Wilms tumor genetics". 502:Characterized by macrosmia (large birth size), 2896: 2650: 2242: 2240: 2135: 2133: 2131: 2129: 2127: 2125: 2123: 2121: 2119: 2117: 728:of tumor cells (fresh or paraffin-embedded). 327:Wilms' tumor is a malignant tumor containing 8: 1937:"Treatment by type and stage of Wilms tumor" 1209: 1207: 1205: 1203: 1175: 1173: 407:. This latter gene is found on short arm of 64:showing the three elements of Wilms' tumor. 1700: 1698: 1696: 1007:Diffuse anaplastic (preoperative treatment) 163:~500 new diagnoses per year (United States) 3310: 2970: 2961: 2903: 2889: 2881: 2686: 2657: 2643: 2635: 2455: 803:+ lymph node sampling followed by regimen 496:Abnormal regulation of chromosome 11p15.5 383:gene which is located on the short arm of 32: 2359: 2272: 2097: 1863: 1730: 1676: 1350: 1300: 920:Focal anaplastic (preoperative treatment) 1449:Journal of Pediatric Hematology/Oncology 737: 518:(enlargement of organs inside abdomen). 416: 373:Molecular biology and related conditions 1169: 403:, the gene encoding the proto-oncogene 1800: 1790: 1833: 1831: 1816: 1814: 1762: 1760: 678:Tumor is present at surgical margins. 7: 1587:American Journal of Medical Genetics 1410: 1408: 1406: 1082:Preoperative treatment with regimen 1064:Preoperative treatment with regimen 1046:Preoperative treatment with regimen 27:Rare childhood cancer of the kidneys 2942:Desmoplastic small-round-cell tumor 2630:Cancer.Net Wilms' Tumor – Childhood 2160:Expert Review of Anticancer Therapy 1856:10.3978/j.issn.2224-4336.2014.01.09 1239:"Wilms tumor: MedlinePlus Genetics" 228:a painless, palpable abdominal mass 1912:"Wilms Tumor - Childhood - Stages" 1773:. National Cancer Institute (US). 1423:, National Cancer Institute (US), 704:are as a whole placed in Stage V. 25: 3019:Aggressive infantile fibromatosis 2690:Glandular and epithelial neoplasm 2203:Nature Clinical Practice. Urology 440:Gene deletion that includes both 3587:Diseases named after discoverers 3508:Clear-cell sarcoma of the kidney 2730:Clear-cell sarcoma of the kidney 1421:PDQ Cancer Information Summaries 1146:National Wilms Tumor Study Group 395:, while a minority are acquired 80: 51: 3276:Intradermal spindle cell lipoma 2987:Dermatofibrosarcoma protuberans 1657:Genes, Chromosomes & Cancer 3104:Malignant fibrous histiocytoma 3052:Infantile digital fibromatosis 3038:Familial myxovascular fibromas 3034:Diffuse infantile fibromatosis 2870:Malignant fibrous histiocytoma 2301:Medical and Pediatric Oncology 1271:Mount Sinai Hospital, New York 457:Characterized by Wilms tumor, 1: 3150:Superficial acral fibromyxoma 3057:Juvenile hyaline fibromatosis 2265:10.1158/1078-0432.CCR-17-0710 1975:10.1158/1078-0432.CCR-07-1081 1289:Annals of 3D Printed Medicine 3572:Small-blue-round-cell tumors 3451:Solitary cutaneous leiomyoma 3441:Multiple cutaneous leiomyoma 3162:Ossifying fibromyxoid tumour 2340:Pediatric Blood & Cancer 2086:Journal of Clinical Oncology 2007:Journal of Clinical Oncology 1888:"How is Wilms tumor staged?" 1711:Journal of Clinical Oncology 1556:10.1016/0092-8674(90)90601-A 1461:10.1097/MPH.0b013e3181e5e20d 717: 532:located on the short arm of 528:has been reported. H19 is a 3099:Benign fibrous histiocytoma 2839:Transitional cell carcinoma 2811:Transitional cell carcinoma 2147:. Last Modified: 03/29/2012 613:and is completely excised . 492:Beckwith-Wiedemann Syndrome 423:Associated Genetic Variant 3603: 3379:Embryonal rhabdomyosarcoma 2957:Connective tissue neoplasm 2766:Juxtaglomerular cell tumor 2055:10.1016/j.ejca.2009.01.005 2043:European Journal of Cancer 1502:10.15586/codon.wt.2016.ch1 1302:10.1016/j.stlm.2022.100077 1105:tumor in individuals with 514:(open abdominal wall) and 471:Denys-Drash syndrome (DDS) 155:~90% of children are cured 3503:Malignant rhabdoid tumour 3470:Complex mixed and stromal 3399: 3391:Alveolar rhabdomyosarcoma 2761:Renal medullary carcinoma 2625:National Cancer Institute 2145:National Cancer Institute 2019:10.1200/jco.2001.19.2.488 1390:WhoNamedIt.com: Max Wilms 1219:National Cancer Institute 1020: 961: 885: 834: 773: 672:Inoperable primary tumor. 200:that typically occurs in 59: 50: 3042:Fibroma of tendon sheath 2253:Clinical Cancer Research 2141:Treatment of Wilms Tumor 2099:10.1200/JCO.2005.01.2799 1963:Clinical Cancer Research 1844:Translational Pediatrics 1723:10.1200/JCO.2015.62.1888 1125:computed tomography scan 912:100% (8 people in study) 609:Tumor is limited to the 567:computed tomography scan 429:Description of Syndrome 3072:Oral submucous fibrosis 3014:Aggressive fibromatosis 2930:Not otherwise specified 2844:Squamous-cell carcinoma 2816:Squamous-cell carcinoma 2771:Renal medullary fibroma 1352:10.1038/nrurol.2017.163 708:Treatment and prognosis 246:(in about 20% of cases) 3114:Solitary fibrous tumor 3109:Atypical fibroxanthoma 2313:10.1002/mpo.2950210305 1339:Nature Reviews Urology 675:Lymph node metastasis. 558: 284: 272: 3488:Mixed Müllerian tumor 1969:(22 Pt 1): 6593–602. 753:relapse-free survival 718:staging and treatment 553: 426:Risk for Wilms tumor 278: 270: 3493:Mesoblastic nephroma 3062:Plantar fibromatosis 2992:Desmoplastic fibroma 2725:Mesoblastic nephroma 2697:Renal cell carcinoma 524:An association with 348:rhabdomyosarcomatous 329:metanephric blastema 293:Denys-Drash syndrome 3483:Pleomorphic adenoma 3291:Spindle cell lipoma 3091:histiocytic sarcoma 3067:Pleomorphic fibroma 3029:Collagenous fibroma 3024:Aponeurotic fibroma 2937:Soft-tissue sarcoma 2745:Metanephric adenoma 2398:1990Natur.346..194P 1037:Favorable histology 965:Favorable histology 889:Favorable histology 838:Favorable histology 757:event-free survival 726:virtual karyotyping 616:The surface of the 461:(absence of iris), 250:high blood pressure 240:nausea and vomiting 60:High magnification 3446:Neural fibrolipoma 3421:Angiolipoleiomyoma 3384:Sarcoma botryoides 3281:Pleomorphic lipoma 3250:Myxoid liposarcoma 3214:Clear-cell sarcoma 3047:Fibromatosis colli 2821:Inverted papilloma 2565:External resources 2215:10.1038/ncpuro1218 2172:10.1586/era.09.159 1159:for 1p and 16q LOH 1073:Diffuse anaplastic 993:Diffuse anaplastic 948:Diffuse anaplastic 934:Diffuse anaplastic 869:Diffuse anaplastic 733:random variability 559: 530:long noncoding RNA 285: 273: 244:blood in the urine 220:Signs and symptoms 3567:Pediatric cancers 3554: 3553: 3546:Adenomatoid tumor 3518:Pancreatoblastoma 3464: 3463: 3426:Genital leiomyoma 3286:Lipoblastomatosis 3222: 3221: 3122: 3121: 3077:Pachydermodactyly 2878: 2877: 2793:Ureteral neoplasm 2779: 2778: 2602: 2601: 2352:10.1002/pbc.20891 2259:(13): e115–e122. 1717:(27): 2999–3007. 1669:10.1002/gcc.20553 1496:. pp. 3–21. 1157:Virtual Karyotype 1090: 1089: 551: 522: 521: 397:somatic mutations 377:Mutations of the 179: 178: 30:Medical condition 18:Wilm's tumor 16:(Redirected from 3594: 3375:rhabdomyosarcoma 3364: 3340: 3316: 3311: 3271:Chondroid lipoma 3209:Synovial sarcoma 3145:Cutaneous myxoma 2971: 2962: 2905: 2898: 2891: 2882: 2702:Renal oncocytoma 2687: 2659: 2652: 2645: 2636: 2607:Wilms' tumor 2456: 2444: 2441: 2435: 2432: 2426: 2425: 2406:10.1038/346194a0 2380: 2374: 2373: 2363: 2331: 2325: 2324: 2296: 2287: 2286: 2276: 2244: 2235: 2234: 2198: 2192: 2191: 2154: 2148: 2137: 2112: 2111: 2101: 2073: 2067: 2066: 2037: 2031: 2030: 2001: 1995: 1994: 1957: 1951: 1950: 1948: 1947: 1933: 1927: 1926: 1924: 1923: 1908: 1902: 1901: 1899: 1898: 1884: 1878: 1877: 1867: 1835: 1826: 1825: 1818: 1809: 1808: 1802: 1798: 1796: 1788: 1786: 1785: 1764: 1755: 1751: 1745: 1744: 1734: 1702: 1691: 1690: 1680: 1648: 1642: 1641: 1617: 1611: 1610: 1582: 1576: 1575: 1538: 1532: 1531: 1487: 1481: 1480: 1444: 1438: 1437: 1436: 1435: 1412: 1401: 1398: 1392: 1387: 1381: 1380: 1354: 1329: 1323: 1322: 1304: 1279: 1273: 1269:; accessed from 1260: 1254: 1253: 1251: 1249: 1235: 1229: 1228: 1226: 1225: 1211: 1198: 1197: 1195: 1193: 1177: 1152:Perlman syndrome 1055:Focal anaplastic 979:Focal anaplastic 906:Focal anaplastic 852:Focal anaplastic 764:overall survival 738: 552: 506:(large tongue), 417: 231:loss of appetite 188:, also known as 103: 102: 99: 98: 95: 92: 89: 86: 55: 33: 21: 3602: 3601: 3597: 3596: 3595: 3593: 3592: 3591: 3557: 3556: 3555: 3550: 3527: 3460: 3395: 3362:Skeletal muscle 3360: 3355: 3336: 3331: 3314: 3300: 3218: 3194: 3190:Phyllodes tumor 3172:Fibroepithelial 3166: 3118: 3081: 2996: 2951: 2925: 2909: 2879: 2874: 2858: 2825: 2797: 2775: 2749: 2740:Cystic nephroma 2706: 2676: 2674:genital systems 2663: 2603: 2598: 2597: 2560: 2559: 2467: 2453: 2448: 2447: 2442: 2438: 2433: 2429: 2392:(6280): 194–7. 2382: 2381: 2377: 2333: 2332: 2328: 2298: 2297: 2290: 2246: 2245: 2238: 2200: 2199: 2195: 2166:(12): 1807–15. 2156: 2155: 2151: 2138: 2115: 2092:(29): 7312–21. 2075: 2074: 2070: 2039: 2038: 2034: 2003: 2002: 1998: 1959: 1958: 1954: 1945: 1943: 1935: 1934: 1930: 1921: 1919: 1910: 1909: 1905: 1896: 1894: 1886: 1885: 1881: 1837: 1836: 1829: 1820: 1819: 1812: 1799: 1789: 1783: 1781: 1766: 1765: 1758: 1752: 1748: 1704: 1703: 1694: 1650: 1649: 1645: 1632:(22): 6288–92. 1626:Cancer Research 1619: 1618: 1614: 1584: 1583: 1579: 1540: 1539: 1535: 1512: 1489: 1488: 1484: 1446: 1445: 1441: 1433: 1431: 1414: 1413: 1404: 1399: 1395: 1388: 1384: 1345:(12): 743–752. 1331: 1330: 1326: 1281: 1280: 1276: 1261: 1257: 1247: 1245: 1243:medlineplus.gov 1237: 1236: 1232: 1223: 1221: 1213: 1212: 1201: 1191: 1189: 1179: 1178: 1171: 1166: 1141:Hemihypertrophy 1137: 1115: 1098: 714:5-year survival 710: 701: 693: 666: 639: 603: 591: 544: 542: 508:hemihypertrophy 480:(exon 8 and 9) 463:hemihypertrophy 375: 265: 222: 144: 107: 83: 79: 45: 31: 28: 23: 22: 15: 12: 11: 5: 3600: 3598: 3590: 3589: 3584: 3579: 3574: 3569: 3559: 3558: 3552: 3551: 3549: 3548: 3543: 3537: 3535: 3529: 3528: 3526: 3525: 3523:Carcinosarcoma 3520: 3515: 3513:Hepatoblastoma 3510: 3505: 3500: 3495: 3490: 3485: 3480: 3474: 3472: 3466: 3465: 3462: 3461: 3459: 3458: 3453: 3448: 3443: 3438: 3433: 3431:Leiomyosarcoma 3428: 3423: 3418: 3416:Angioleiomyoma 3413: 3412: 3411: 3400: 3397: 3396: 3394: 3393: 3388: 3387: 3386: 3367: 3365: 3357: 3356: 3354: 3353: 3351:leiomyosarcoma 3343: 3341: 3333: 3332: 3330: 3329: 3319: 3317: 3308: 3302: 3301: 3299: 3298: 3293: 3288: 3283: 3278: 3273: 3267: 3266: 3265: 3264: 3262:Angiomyolipoma 3254: 3253: 3252: 3247: 3232: 3230: 3224: 3223: 3220: 3219: 3217: 3216: 3211: 3205: 3203: 3196: 3195: 3193: 3192: 3187: 3182: 3180:Brenner tumour 3176: 3174: 3168: 3167: 3165: 3164: 3159: 3154: 3153: 3152: 3147: 3132: 3130: 3124: 3123: 3120: 3119: 3117: 3116: 3111: 3106: 3101: 3095: 3093: 3083: 3082: 3080: 3079: 3074: 3069: 3064: 3059: 3054: 3049: 3044: 3039: 3036: 3031: 3026: 3021: 3016: 3010: 3008: 2998: 2997: 2995: 2994: 2989: 2983: 2981: 2968: 2959: 2953: 2952: 2950: 2949: 2944: 2939: 2933: 2931: 2927: 2926: 2910: 2908: 2907: 2900: 2893: 2885: 2876: 2875: 2873: 2872: 2866: 2864: 2860: 2859: 2857: 2856: 2851: 2849:Adenocarcinoma 2846: 2841: 2835: 2833: 2827: 2826: 2824: 2823: 2818: 2813: 2807: 2805: 2799: 2798: 2796: 2795: 2789: 2787: 2781: 2780: 2777: 2776: 2774: 2773: 2768: 2763: 2757: 2755: 2751: 2750: 2748: 2747: 2742: 2737: 2735:Angiomyolipoma 2732: 2727: 2722: 2716: 2714: 2708: 2707: 2705: 2704: 2699: 2693: 2691: 2684: 2678: 2677: 2664: 2662: 2661: 2654: 2647: 2639: 2633: 2632: 2627: 2618: 2613: 2600: 2599: 2596: 2595: 2581: 2569: 2568: 2566: 2562: 2561: 2558: 2557: 2546: 2535: 2524: 2510: 2499: 2484: 2468: 2463: 2462: 2460: 2459:Classification 2452: 2451:External links 2449: 2446: 2445: 2436: 2427: 2375: 2326: 2288: 2236: 2209:(10): 551–60. 2193: 2149: 2113: 2068: 2032: 2013:(2): 488–500. 1996: 1952: 1941:www.cancer.org 1928: 1918:. 25 June 2012 1903: 1892:www.cancer.org 1879: 1827: 1810: 1756: 1746: 1692: 1643: 1612: 1577: 1533: 1510: 1482: 1439: 1402: 1393: 1382: 1333:(2017-12-01). 1324: 1283:(2022-10-01). 1274: 1263:EBSCO database 1255: 1230: 1199: 1181:"Wilms' tumor" 1168: 1167: 1165: 1162: 1161: 1160: 1154: 1149: 1143: 1136: 1133: 1114: 1111: 1097: 1094: 1088: 1087: 1080: 1077: 1074: 1070: 1069: 1062: 1059: 1056: 1052: 1051: 1044: 1041: 1038: 1034: 1033: 1031: 1028: 1025: 1022: 1021:Bilateral (V) 1018: 1017: 1014: 1011: 1008: 1004: 1003: 1000: 997: 994: 990: 989: 986: 983: 980: 976: 975: 972: 969: 966: 963: 959: 958: 955: 952: 949: 945: 944: 941: 938: 935: 931: 930: 927: 924: 921: 917: 916: 913: 910: 907: 903: 902: 896: 893: 890: 887: 883: 882: 876: 873: 870: 866: 865: 859: 856: 853: 849: 848: 845: 842: 839: 836: 832: 831: 821: 818: 815: 808: 807: 798: 795: 792: 788: 787: 784: 781: 778: 775: 771: 770: 767: 760: 749: 747:Histopathology 744: 709: 706: 700: 697: 692: 689: 688: 687: 686: 685: 679: 676: 673: 665: 662: 661: 660: 659: 658: 655: 649: 646: 638: 635: 634: 633: 630: 627: 624: 621: 614: 602: 599: 590: 587: 541: 538: 520: 519: 500: 497: 494: 488: 487: 484: 481: 473: 467: 466: 455: 452: 438: 431: 430: 427: 424: 421: 420:Syndrome Name 374: 371: 370: 369: 361: 325: 324: 319: 313: 264: 261: 260: 259: 253: 247: 241: 238: 235: 234:abdominal pain 232: 229: 221: 218: 190:nephroblastoma 177: 176: 171: 165: 164: 161: 157: 156: 153: 147: 146: 141: 137: 136: 133: 129: 128: 115: 109: 108: 106: 105: 76: 74: 70: 69: 57: 56: 48: 47: 46:Nephroblastoma 42: 38: 37: 29: 26: 24: 14: 13: 10: 9: 6: 4: 3: 2: 3599: 3588: 3585: 3583: 3580: 3578: 3577:Kidney cancer 3575: 3573: 3570: 3568: 3565: 3564: 3562: 3547: 3544: 3542: 3539: 3538: 3536: 3534: 3530: 3524: 3521: 3519: 3516: 3514: 3511: 3509: 3506: 3504: 3501: 3499: 3496: 3494: 3491: 3489: 3486: 3484: 3481: 3479: 3476: 3475: 3473: 3471: 3467: 3457: 3454: 3452: 3449: 3447: 3444: 3442: 3439: 3437: 3434: 3432: 3429: 3427: 3424: 3422: 3419: 3417: 3414: 3410: 3407: 3406: 3405: 3402: 3401: 3398: 3392: 3389: 3385: 3382: 3381: 3380: 3376: 3372: 3369: 3368: 3366: 3363: 3358: 3352: 3348: 3345: 3344: 3342: 3339: 3338:Smooth muscle 3334: 3328: 3324: 3321: 3320: 3318: 3312: 3309: 3307: 3303: 3297: 3294: 3292: 3289: 3287: 3284: 3282: 3279: 3277: 3274: 3272: 3269: 3268: 3263: 3260: 3259: 3258: 3255: 3251: 3248: 3246: 3243: 3242: 3241: 3237: 3234: 3233: 3231: 3229: 3225: 3215: 3212: 3210: 3207: 3206: 3204: 3201: 3197: 3191: 3188: 3186: 3183: 3181: 3178: 3177: 3175: 3173: 3169: 3163: 3160: 3158: 3155: 3151: 3148: 3146: 3143: 3142: 3141: 3137: 3134: 3133: 3131: 3129: 3125: 3115: 3112: 3110: 3107: 3105: 3102: 3100: 3097: 3096: 3094: 3092: 3088: 3084: 3078: 3075: 3073: 3070: 3068: 3065: 3063: 3060: 3058: 3055: 3053: 3050: 3048: 3045: 3043: 3040: 3037: 3035: 3032: 3030: 3027: 3025: 3022: 3020: 3017: 3015: 3012: 3011: 3009: 3007: 3003: 2999: 2993: 2990: 2988: 2985: 2984: 2982: 2980: 2976: 2972: 2969: 2967: 2963: 2960: 2958: 2954: 2948: 2945: 2943: 2940: 2938: 2935: 2934: 2932: 2928: 2924: 2920: 2917: 2913: 2906: 2901: 2899: 2894: 2892: 2887: 2886: 2883: 2871: 2868: 2867: 2865: 2861: 2855: 2852: 2850: 2847: 2845: 2842: 2840: 2837: 2836: 2834: 2832: 2828: 2822: 2819: 2817: 2814: 2812: 2809: 2808: 2806: 2804: 2800: 2794: 2791: 2790: 2788: 2786: 2782: 2772: 2769: 2767: 2764: 2762: 2759: 2758: 2756: 2752: 2746: 2743: 2741: 2738: 2736: 2733: 2731: 2728: 2726: 2723: 2721: 2718: 2717: 2715: 2713: 2709: 2703: 2700: 2698: 2695: 2694: 2692: 2688: 2685: 2683: 2679: 2675: 2671: 2667: 2660: 2655: 2653: 2648: 2646: 2641: 2640: 2637: 2631: 2628: 2626: 2622: 2619: 2617: 2614: 2612: 2608: 2605: 2604: 2594: 2591: 2587: 2586: 2582: 2580: 2576: 2575: 2571: 2570: 2567: 2563: 2556: 2552: 2551: 2547: 2545: 2541: 2540: 2536: 2534: 2530: 2529: 2525: 2523: 2520: 2516: 2515: 2511: 2509: 2505: 2504: 2500: 2498: 2494: 2493: 2489: 2485: 2483: 2479: 2478: 2474: 2470: 2469: 2466: 2461: 2457: 2450: 2440: 2437: 2431: 2428: 2423: 2419: 2415: 2411: 2407: 2403: 2399: 2395: 2391: 2387: 2379: 2376: 2371: 2367: 2362: 2357: 2353: 2349: 2345: 2341: 2337: 2330: 2327: 2322: 2318: 2314: 2310: 2307:(3): 172–81. 2306: 2302: 2295: 2293: 2289: 2284: 2280: 2275: 2270: 2266: 2262: 2258: 2254: 2250: 2243: 2241: 2237: 2232: 2228: 2224: 2220: 2216: 2212: 2208: 2204: 2197: 2194: 2189: 2185: 2181: 2177: 2173: 2169: 2165: 2161: 2153: 2150: 2146: 2142: 2136: 2134: 2132: 2130: 2128: 2126: 2124: 2122: 2120: 2118: 2114: 2109: 2105: 2100: 2095: 2091: 2087: 2083: 2079: 2072: 2069: 2064: 2060: 2056: 2052: 2049:(5): 819–26. 2048: 2044: 2036: 2033: 2028: 2024: 2020: 2016: 2012: 2008: 2000: 1997: 1992: 1988: 1984: 1980: 1976: 1972: 1968: 1964: 1956: 1953: 1942: 1938: 1932: 1929: 1917: 1913: 1907: 1904: 1893: 1889: 1883: 1880: 1875: 1871: 1866: 1861: 1857: 1853: 1849: 1845: 1841: 1834: 1832: 1828: 1823: 1817: 1815: 1811: 1806: 1794: 1780: 1776: 1772: 1771: 1763: 1761: 1757: 1750: 1747: 1742: 1738: 1733: 1728: 1724: 1720: 1716: 1712: 1708: 1701: 1699: 1697: 1693: 1688: 1684: 1679: 1674: 1670: 1666: 1663:(6): 461–70. 1662: 1658: 1654: 1647: 1644: 1639: 1635: 1631: 1627: 1623: 1616: 1613: 1608: 1604: 1600: 1596: 1592: 1588: 1581: 1578: 1573: 1569: 1565: 1561: 1557: 1553: 1550:(3): 509–20. 1549: 1545: 1537: 1534: 1529: 1525: 1521: 1517: 1513: 1511:9780994438119 1507: 1503: 1499: 1495: 1494: 1486: 1483: 1478: 1474: 1470: 1466: 1462: 1458: 1454: 1450: 1443: 1440: 1430: 1426: 1422: 1418: 1411: 1409: 1407: 1403: 1397: 1394: 1391: 1386: 1383: 1378: 1374: 1370: 1366: 1362: 1358: 1353: 1348: 1344: 1340: 1336: 1328: 1325: 1320: 1316: 1312: 1308: 1303: 1298: 1294: 1290: 1286: 1278: 1275: 1272: 1268: 1264: 1259: 1256: 1244: 1240: 1234: 1231: 1220: 1216: 1210: 1208: 1206: 1204: 1200: 1188: 1187: 1182: 1176: 1174: 1170: 1163: 1158: 1155: 1153: 1150: 1147: 1144: 1142: 1139: 1138: 1134: 1132: 1130: 1129:Mario Costici 1126: 1121: 1119: 1118:Sidney Farber 1112: 1110: 1108: 1102: 1095: 1093: 1085: 1081: 1078: 1075: 1072: 1071: 1067: 1063: 1060: 1057: 1054: 1053: 1049: 1045: 1042: 1039: 1036: 1035: 1032: 1029: 1026: 1023: 1019: 1015: 1012: 1009: 1006: 1005: 1001: 998: 995: 992: 991: 987: 984: 981: 978: 977: 973: 970: 967: 964: 960: 956: 953: 950: 947: 946: 942: 939: 936: 933: 932: 928: 925: 922: 919: 918: 914: 911: 908: 905: 904: 901: 897: 894: 891: 888: 884: 881: 877: 874: 871: 868: 867: 864: 860: 857: 854: 851: 850: 846: 843: 840: 837: 833: 830: 826: 822: 819: 816: 814: 810: 809: 806: 802: 799: 796: 793: 790: 789: 785: 782: 779: 776: 772: 768: 765: 761: 758: 754: 750: 748: 745: 743: 740: 739: 736: 734: 729: 727: 721: 719: 715: 707: 705: 698: 696: 690: 683: 682: 680: 677: 674: 671: 670: 669: 663: 656: 653: 652: 650: 647: 644: 643: 642: 636: 631: 628: 625: 622: 619: 618:renal capsule 615: 612: 608: 607: 606: 600: 598: 596: 588: 586: 584: 581:or in Europe 580: 576: 572: 568: 563: 556: 539: 537: 535: 534:chromosome 11 531: 527: 517: 516:visceromegaly 513: 509: 505: 501: 498: 495: 493: 490: 489: 485: 482: 479: 478: 474: 472: 469: 468: 464: 460: 456: 453: 451: 450: 445: 444: 439: 436: 435:WAGR syndrome 433: 432: 428: 425: 422: 419: 418: 415: 412: 410: 406: 402: 398: 394: 390: 386: 385:chromosome 11 382: 381: 372: 367: 366: 362: 359: 356: 355: 354: 351: 349: 344: 342: 338: 334: 330: 323: 320: 317: 314: 312: 309: 308: 307: 304: 302: 298: 294: 290: 289:Wilms Tumor 1 282: 281:H&E stain 277: 269: 262: 258: 254: 251: 248: 245: 242: 239: 236: 233: 230: 227: 226: 225: 219: 217: 216:being cured. 213: 211: 207: 203: 199: 195: 191: 187: 183: 175: 172: 170: 166: 162: 158: 154: 152: 148: 142: 138: 135:1–4 years old 134: 130: 127: 123: 119: 116: 114: 110: 101: 78: 77: 75: 73:Pronunciation 71: 67: 66:H&E stain 63: 58: 54: 49: 43: 39: 34: 19: 3582:Rare cancers 3541:Mesothelioma 3498:Wilms' tumor 3497: 3185:Fibroadenoma 3087:Histiocytoma 3006:fibromatosis 2979:fibrosarcoma 2947:Skin sarcoma 2720:Wilms' tumor 2719: 2583: 2572: 2548: 2537: 2526: 2512: 2501: 2486: 2471: 2439: 2430: 2389: 2385: 2378: 2346:(3): 260–7. 2343: 2339: 2329: 2304: 2300: 2256: 2252: 2206: 2202: 2196: 2163: 2159: 2152: 2089: 2085: 2071: 2046: 2042: 2035: 2010: 2006: 1999: 1966: 1962: 1955: 1944:. Retrieved 1940: 1931: 1920:. Retrieved 1915: 1906: 1895:. Retrieved 1891: 1882: 1850:(1): 12–24. 1847: 1843: 1782:. Retrieved 1769: 1749: 1714: 1710: 1660: 1656: 1646: 1629: 1625: 1615: 1593:(4): 260–7. 1590: 1586: 1580: 1547: 1543: 1536: 1492: 1485: 1455:(6): 486–8. 1452: 1448: 1442: 1432:, retrieved 1420: 1396: 1385: 1342: 1338: 1327: 1292: 1288: 1277: 1265:verified by 1258: 1246:. Retrieved 1242: 1233: 1222:. Retrieved 1218: 1190:. Retrieved 1184: 1122: 1116: 1103: 1099: 1096:Epidemiology 1091: 829:radiotherapy 730: 722: 712:The overall 711: 702: 694: 667: 640: 604: 592: 583:chemotherapy 564: 560: 523: 504:macroglossia 475: 447: 441: 413: 409:chromosome 3 405:beta-catenin 378: 376: 363: 357: 352: 345: 326: 305: 286: 263:Pathogenesis 223: 214: 189: 185: 182:Wilms' tumor 181: 180: 145:Radiotherapy 44:Wilms' tumor 36:Wilms' tumor 3533:Mesothelial 3371:Rhabdomyoma 3245:Myelolipoma 3240:liposarcoma 3157:Angiomyxoma 3140:myxosarcoma 2966:Fibromatous 2916:soft tissue 2754:by location 2712:Mixed tumor 2621:Information 2574:MedlinePlus 2076:Grundy PE, 1801:|work= 1186:Mayo Clinic 1123:The use of 801:Nephrectomy 579:nephrectomy 536:(11p15.5). 512:omphalocele 204:(rarely in 186:Wilms tumor 169:Named after 143:Nephrectomy 132:Usual onset 41:Other names 3561:Categories 3478:Adenomyoma 3327:myosarcoma 3228:Lipomatous 3128:Myxomatous 2912:Connective 2539:DiseasesDB 2078:Breslow NE 1946:2015-11-13 1922:2015-11-15 1916:Cancer.Net 1897:2015-11-15 1784:2018-11-12 1434:2018-11-26 1295:: 100077. 1224:2018-11-12 1164:References 813:anaplastic 769:Treatment 620:is intact. 595:anatomical 411:(3p22.1). 365:Anaplastic 322:epithelium 316:mesenchyme 297:metastasis 257:varicocele 255:Rarely as 126:nephrology 62:micrograph 3409:Cutaneous 3404:Leiomyoma 3347:Leiomyoma 3306:Myomatous 3296:Hibernoma 2585:eMedicine 2555:302849000 2550:SNOMED CT 2188:207212698 1803:ignored ( 1793:cite book 1477:205860918 1361:1759-4820 1319:251870073 1311:2666-9641 1192:March 10, 880:regimen I 755:(RFS) or 699:Bilateral 664:Stage III 540:Diagnosis 391:from the 389:inherited 358:Favorable 337:cartilage 210:Max Wilms 174:Max Wilms 160:Frequency 151:Prognosis 140:Treatment 113:Specialty 3200:Synovial 2923:sarcomas 2854:Melanoma 2593:ped/2440 2590:med/3093 2370:16700047 2283:28674120 2231:23599363 2223:18836464 2180:19954292 2108:16129848 2063:19231157 2027:11208843 1991:17036977 1983:18006759 1874:26835318 1779:26389282 1741:26304882 1687:18311776 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Index

Wilm's tumor

micrograph
H&E stain
/vɪlmz/
Specialty
Oncology
urology
nephrology
Prognosis
Named after
Max Wilms
cancer
kidneys
children
adults
Max Wilms
blood in the urine
high blood pressure
varicocele


H&E stain
Wilms Tumor 1
Denys-Drash syndrome
metastasis
bleeding
blastema
mesenchyme
epithelium

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