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| '''For patient information click [[Kidney cancer (patient information)|here]]''' | | __NOTOC__ |
| | {{Wilms' tumor}} |
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| {{Infobox_Disease | | | {{CMG}}; {{AE}} {{SSW}},[[User:Savni Satoskar|Savni Satoskar]], {{SC}} |
| Name = {{PAGENAME}} |
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| Image = Wilms tm.jpg |
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| Caption = |
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| DiseasesDB = 8896 |
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| ICD10 = {{ICD10|C|64||c|64}}|
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| ICD9 = {{ICD9|189.0}} |
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| ICDO = {{ICDO|8960|3}} |
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| OMIM = 194070 |
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| OMIM_mult = {{OMIM2|607102}} |
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| MedlinePlus = |
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| eMedicineSubj = med |
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| eMedicineTopic = 3093 |
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| eMedicine_mult = {{eMedicine2|ped|2440}} |
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| MeshID = D009396 |
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| }}
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| {{Search infobox}}
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| {{SCC}} | |
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| {{Editor Join}}
| | '''For patient information click [[Wilms' tumor (patient information)|here]]''' |
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| ==Overview==
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| '''Wilms' tumor''' or '''nephroblastoma''' is a [[tumor]] of the [[kidney]]s that typically occurs in children, rarely in [[adult]]s. Its common name is an [[eponym]], referring to Dr. [[Max Wilms]], the German surgeon (1867-1918) who first described this kind of tumor.<ref>[http://www.whonamedit.com/doctor.cfm/2109.html WhoNamedIt.com: Max Wilms]</ref>
| | {{SK}} Nephroblastoma |
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| Approximately 500 cases are diagnosed in the U.S. annually. The majority (75%) occur in otherwise normal children; a minority (25%) is associated with other developmental abnormalities. It is highly responsive to treatment, with about 90% of patients surviving at least five years.
| | ==[[Wilms' tumor overview|Overview]]== |
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| == Pathology == | | ==[[Wilms' tumor historical perspective|Historical Perspective]]== |
| Pathologically, a triphasic nephroblastoma comprises three elements:
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| * [[blastema]]
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| * [[mesenchyme]]
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| * [[epithelium]]
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| Wilms' tumor is a malignant tumor containing [[metanephric blastema]], stromal and epithelial derivatives. Characteristic is the presence of abortive tubules and glomeruli surrounded by a spindled cell stroma. The stroma may include striated [[muscle]], [[cartilage]], [[bone]], fat tissue, fibrous tissue. The tumor is compressing the normal kidney parenchyma. [http://www.pathologyatlas.ro/Wilms%20Tumor.html Pathology images] | | ==[[Wilms' tumor pathophysiology|Pathophysiology]]== |
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| The mesenchymal component may include cells showing rhabdomyoid differentiation. The rhabdomyoid component may itself show features of malignancy ([[rhabdomyosarcoma]]tous Wilms).
| | ==[[Wilms' tumor causes|Causes]]== |
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| Wilms tumor may be separated into 2 prognostic groups based on pathologic characteristics: | | ==[[Wilms' tumor differentiating from other disease|Differentiating Wilms' tumor from other Diseases]]== |
| * ''Favorable'' - Contains well developed components mentioned above
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| * ''[[Anaplastic]]'' - Contains diffuse anaplasia (poorly developed cells)
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| == Molecular biology == | | ==[[Wilms' tumor epidemiology and demographics|Epidemiology and Demographics]]== |
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| Mutations of the [[WT1]] gene on chromosome 11 are observed in approximately 20% of Wilms' tumors.<ref>{{cite journal |author=Call K, Glaser T, Ito C, Buckler A, Pelletier J, Haber D, Rose E, Kral A, Yeger H, Lewis W |title=Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locus |journal=Cell |volume=60 |issue=3 |pages=509-20 |year=1990 |pmid=2154335}}</ref><ref>{{cite journal |author=Huff V |title=Wilms tumor genetics |journal=Am J Med Genet |volume=79 |issue=4 |pages=260-7 |year=1998 |pmid=9781905}}</ref> At least half of the Wilms' tumors with mutations in WT1 also carry mutations in CTNNB1, the gene encoding the proto-oncogene beta-catenin.<ref>{{cite journal |author=Maiti S, Alam R, Amos CI, Huff V |title=Frequent association of beta-catenin and WT1 mutations in Wilms tumors |journal=Cancer Res |volume=60 |issue=22 |pages=6288-92 |year=2000 |pmid=11103785}}</ref>
| | ==[[Wilms' tumor risk factors|Risk Factors]]== |
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| A gene on the X chromosome, WTX, is inactivated in up to 30% of Wilms' tumor cases, according to research published in 2007.<ref>{{cite journal |author=Rivera M, Kim W, Wells J, Driscoll D, Brannigan B, Han M, Kim J, Feinberg A, Gerald W, Vargas S, Chin L, Iafrate A, Bell D, Haber D |title=An X chromosome gene, WTX, is commonly inactivated in Wilms tumor |journal=Science |volume=315 |issue=5812 |pages=642-5 |year=2007 |pmid=17204608}}</ref>
| | ==[[Wilms' tumor screening|Screening]]== |
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| | ==[[Wilms' tumor natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==Diagnosis== | | ==Diagnosis== |
| | [[Wilms' tumor staging|Staging]] | [[Wilms' tumor history and symptoms|History and Symptoms]] | [[Wilms' tumor physical examination|Physical Examination]] | [[Wilms' tumor laboratory findings|Laboratory Findings]] | [[x ray|X Ray]] | [[Wilms' tumor CT|CT]] | [[Wilms' tumor MRI|MRI]] | [[Wilms' tumor ultrasound|Ultrasound]] | [[Wilms' tumor other diagnostic studies|Other Diagnostic Studies]] |
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| ===CT=== | | ==Treatment== |
| [[Image:Wilms Tumor CTScan.gif|frame|left|[[Computed tomography|CT Scan]] of 11 cm '''Wilms' tumor''' of left kidney in 13 month old patient.]] | | [[Wilms' tumor medical therapy|Medical Therapy]] | [[Wilms' tumor surgery|Surgery]] | [[Wilms' tumor cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Wilms' tumor future or investigational therapies|Future or Investigational Therapies]] |
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| == Staging and treatment ==
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| Staging is determined by combination of imaging studies, and pathologic findings if the tumor is operable (adapted from www.cancer.gov). Treatment strategy is determined by the stage:
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| ===Stage I (43% of patients) ===
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| For stage I Wilms' tumor, 1 or more of the following criteria must be met:
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| * Tumor is limited to the kidney and is completely excised.
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| * The surface of the renal capsule is intact.
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| * The tumor is not ruptured or biopsied (open or needle) prior to removal.
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| * No involvement of renal sinus vessels.
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| * No residual tumor apparent beyond the margins of excision.
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| Treatment: [[Nephrectomy]] + 18 weeks of [[chemotherapy]]
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| Outcome: 98% 4-year survival; 85% 4-year survival if [[anaplastic]]
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| ===Stage II (23% of patients)=== | | ==Case Studies== |
| For Stage II Wilms' tumor, 1 or more of the following criteria must be met:
| | :[[Wilms' tumor case study one|Case #1]] |
| * Tumor extends beyond the kidney but is completely excised.
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| * No residual tumor apparent at or beyond the margins of excision.
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| * Any of the following conditions may also exist:
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| ** Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma.
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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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| Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy
| | ==Related Chapters== |
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| Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic
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| ===Stage III (23% of patients)=== | |
| For Stage III Wilms' tumor, 1 or more of the following criteria must be met:
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| * Unresectable primary tumor.
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| * Lymph node metastasis.
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| * Positive surgical margins.
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| * Tumor spillage involving peritoneal surfaces either before or during surgery, or transected tumor thrombus.
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| Treatment: Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor shrinkage
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| Outcome: 95% 4-year survival; 56% 4-year survival if anaplastic
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| ===Stage IV (10% of patients) ===
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| Stage IV Wilms' tumor is defined as the presence of hematogenous metastases (lung, liver, bone, or brain), or lymph node metastases outside the abdomenopelvic region.
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| Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy + radiation of metastatic site as appropriate
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| Outcome: 90% 4-year survival; 17% 4-year survival if anaplastic
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| ===Stage V (5% of patients) ===
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| Stage V Wilms’ tumor is defined as bilateral renal involvement at the time of initial diagnosis.
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| Note: For patients with bilateral involvement, 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. The 4-year survival was 94% for those patients whose most advanced lesion was stage I or stage II; 76% for those whose most advanced lesion was stage III.
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| Treatment: Individualized thereapy based on tumor burden
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| ===Stage I-IV Anaplasia===
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| Children with stage I anaplastic tumors have an excellent prognosis (80-90% five-year survival). They can be managed with the same regimen given to stage I favorable histology patients.
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| Children with stage II through stage IV diffuse anaplasia, however, represent a higher-risk group. These tumors are more resistant to the chemotherapy traditionally used in children with Wilms’ tumor (favorable histology), and require more aggressive regimens.
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| == Treatment ==
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| Once a kidney tumor is found, surgery can find out whether or not the tumor is cancer. A sample of tissue from the tumor is sent to a pathologist, who looks at it under a microscope to check for signs of cancer. If the tumor is only in the kidney, it can be removed along with the whole kidney (a process called [[nephrectomy]]). If there are tumors in both kidneys or if the tumor has spread outside the kidney, a piece of the tumor will be removed.
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| ==See also==
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| *[[National Wilms Tumor Study Group]] (NWTS) | | *[[National Wilms Tumor Study Group]] (NWTS) |
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| ==External links== | | ==External links== |
| * {{cite journal |author=Metzger ML, Dome JS |title=Current therapy for Wilms' tumor |journal=Oncologist |volume=10 |issue=10 |pages=815–26 |year=2005 |pmid=16314292 |doi=10.1634/theoncologist.10-10-815 |url=http://theoncologist.alphamedpress.org/cgi/content/full/10/10/815?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=wilms&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT}}
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| *[http://www.cancer.gov/cancertopics/pdq/treatment/wilms/Patient Information] from [[National Cancer Institute]]
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| *[http://www.emedicine.com/med/topic3093.htm Information] from [[WebMD]]
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| *[http://www.mayoclinic.com/health/wilms-tumor/DS00436 Information] from the [[Mayo Clinic]]
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| *[http://www.kids-cancer.org/wilms.htm Information] from [[Sydney Children's Hospital]] (Australia)
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| *[http://www.pathologyatlas.ro/Wilms%20Tumor.html Photos from Atlas of Pathology]
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| *[http://www.nlm.nih.gov/medlineplus/wilmstumor.html List of additional resources] compiled by the [[NIH]] | | *[http://www.nlm.nih.gov/medlineplus/wilmstumor.html List of additional resources] compiled by the [[NIH]] |
| *{{DMOZ|Health/Conditions_and_Diseases/Cancer/Genitourinary/Kidney/Wilm's_Tumor/}}
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| ==References==
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| {{reflist|2}}
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| {{Nephrology}} | | {{Nephrology}} |
| {{tumors}} | | {{tumors}} |
| {{Soft tissue tumors and sarcomas}} | | {{Soft tissue tumors and sarcomas}} |
| {{SIB}}
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| [[Category:Kidney diseases]] | | [[Category:Kidney diseases]] |
| [[Category:Types of cancer]] | | [[Category:Types of cancer]] |
| | [[Category:Disease]] |
| [[Category:Urology]] | | [[Category:Urology]] |
| [[Category:Pediatrics]] | | [[Category:Pediatrics]] |
| | [[Category:Hematology]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| [[Category:Hematology]]
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| [[de:Nephroblastom]]
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| [[fr:Tumeur de Wilms]]
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| [[es:Tumor de Wilms]]
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| [[hu:Wilms-tumor]]
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| [[nl:Nefroblastoom]]
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| [[pl:Nephroblastoma]]
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| [[pt:Tumor de Wilms]]
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| [[ru:Нефробластома]]
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| [[fi:Wilmsin kasvain]]
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