|
|
| (33 intermediate revisions by 10 users not shown) |
| Line 1: |
Line 1: |
| '''For patient information click [[Wilms' tumor (patient information)|here]]'''
| | __NOTOC__ |
| | |
| {{Infobox_Disease |
| |
| Name = {{PAGENAME}} |
| |
| Image = Wilms tm.jpg |
| |
| Caption = |
| |
| DiseasesDB = 8896 |
| |
| ICD10 = {{ICD10|C|64||c|64}}|
| |
| ICD9 = {{ICD9|189.0}} |
| |
| ICDO = {{ICDO|8960|3}} |
| |
| OMIM = 194070 |
| |
| OMIM_mult = {{OMIM2|607102}} |
| |
| MedlinePlus = |
| |
| MeshID = D009396 |
| |
| }}
| |
| {{Wilms' tumor}} | | {{Wilms' tumor}} |
| {{SCC}}
| |
|
| |
|
| {{Editor Join}} | | {{CMG}}; {{AE}} {{SSW}},[[User:Savni Satoskar|Savni Satoskar]], {{SC}} |
|
| |
|
| ==[[Wilms' tumor overview|Overview]]==
| | '''For patient information click [[Wilms' tumor (patient information)|here]]''' |
|
| |
|
| == Pathology ==
| | {{SCC}}; |
| Pathologically, a triphasic nephroblastoma comprises three elements:
| |
| * [[blastema]]
| |
| * [[mesenchyme]]
| |
| * [[epithelium]]
| |
|
| |
|
| 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]
| | {{SK}} Nephroblastoma |
|
| |
|
| The mesenchymal component may include cells showing rhabdomyoid differentiation. The rhabdomyoid component may itself show features of malignancy ([[rhabdomyosarcoma]]tous Wilms).
| | ==[[Wilms' tumor overview|Overview]]== |
| | |
| Wilms tumor may be separated into 2 prognostic groups based on pathologic characteristics: | |
| * ''Favorable'' - Contains well developed components mentioned above
| |
| * ''[[Anaplastic]]'' - Contains diffuse anaplasia (poorly developed cells)
| |
|
| |
|
| == Molecular biology == | | ==[[Wilms' tumor historical perspective|Historical Perspective]]== |
|
| |
|
| 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 pathophysiology|Pathophysiology]]== |
|
| |
|
| 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 causes|Causes]]== |
|
| |
|
| ==Diagnosis== | | ==[[Wilms' tumor differentiating from other disease|Differentiating Wilms' tumor from other Diseases]]== |
|
| |
|
| ===CT=== | | ==[[Wilms' tumor epidemiology and demographics|Epidemiology and Demographics]]== |
| [[Image:Wilms Tumor CTScan.gif|frame|left|[[Computed tomography|CT Scan]] of 11 cm '''Wilms' tumor''' of left kidney in 13 month old patient.]] | |
| <br clear="left"/>
| |
| == Staging and treatment ==
| |
|
| |
|
| 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:
| | ==[[Wilms' tumor risk factors|Risk Factors]]== |
|
| |
|
| ===Stage I (43% of patients) === | | ==[[Wilms' tumor screening|Screening]]== |
| For stage I Wilms' tumor, 1 or more of the following criteria must be met:
| |
| * Tumor is limited to the kidney and is completely excised.
| |
| * The surface of the renal capsule is intact.
| |
| * The tumor is not ruptured or biopsied (open or needle) prior to removal.
| |
| * No involvement of renal sinus vessels.
| |
| * No residual tumor apparent beyond the margins of excision.
| |
|
| |
|
| Treatment: [[Nephrectomy]] + 18 weeks of [[chemotherapy]]
| | ==[[Wilms' tumor natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
|
| |
| Outcome: 98% 4-year survival; 85% 4-year survival if [[anaplastic]]
| |
|
| |
|
| ===Stage II (23% of patients)=== | | ==Diagnosis== |
| For Stage II Wilms' tumor, 1 or more of the following criteria must be met:
| | [[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]] |
| * Tumor extends beyond the kidney but is completely excised.
| |
| * No residual tumor apparent at or beyond the margins of excision.
| |
| * Any of the following conditions may also exist:
| |
| ** Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma.
| |
| ** The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank.
| |
| | |
| Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy
| |
| | |
| Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic
| |
|
| |
|
| ===Stage III (23% of patients)=== | | ==Treatment== |
| For Stage III Wilms' tumor, 1 or more of the following criteria must be met:
| | [[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]] |
| * Unresectable primary tumor.
| |
| * Lymph node metastasis.
| |
| * Positive surgical margins.
| |
| * Tumor spillage involving peritoneal surfaces either before or during surgery, or transected tumor thrombus.
| |
|
| |
|
| Treatment: Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor shrinkage
| | ==Case Studies== |
| | :[[Wilms' tumor case study one|Case #1]] |
|
| |
|
| Outcome: 95% 4-year survival; 56% 4-year survival if anaplastic
| | ==Related Chapters== |
| | |
| ===Stage IV (10% of patients) === | |
| 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.
| |
| | |
| Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy + radiation of metastatic site as appropriate
| |
| | |
| Outcome: 90% 4-year survival; 17% 4-year survival if anaplastic
| |
| | |
| ===Stage V (5% of patients) ===
| |
| Stage V Wilms’ tumor is defined as bilateral renal involvement at the time of initial diagnosis.
| |
| 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.
| |
|
| |
| Treatment: Individualized thereapy based on tumor burden
| |
| | |
| ===Stage I-IV Anaplasia===
| |
| 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.
| |
| | |
| 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.
| |
| | |
| == Treatment ==
| |
| 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.
| |
| | |
| ==See also==
| |
| *[[National Wilms Tumor Study Group]] (NWTS) | | *[[National Wilms Tumor Study Group]] (NWTS) |
|
| |
|
| ==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}}
| |
| *[http://www.cancer.gov/cancertopics/pdq/treatment/wilms/Patient Information] from [[National Cancer Institute]]
| |
| *[http://www.emedicine.com/med/topic3093.htm Information] from [[WebMD]]
| |
| *[http://www.mayoclinic.com/health/wilms-tumor/DS00436 Information] from the [[Mayo Clinic]]
| |
| *[http://www.kids-cancer.org/wilms.htm Information] from [[Sydney Children's Hospital]] (Australia)
| |
| *[http://www.pathologyatlas.ro/Wilms%20Tumor.html Photos from Atlas of Pathology]
| |
| *[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/}}
| |
|
| |
| ==References==
| |
| {{reflist|2}}
| |
|
| |
|
| {{Nephrology}} | | {{Nephrology}} |
| {{tumors}} | | {{tumors}} |
| {{Soft tissue tumors and sarcomas}} | | {{Soft tissue tumors and sarcomas}} |
| {{SIB}}
| |
|
| |
|
| [[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]]
| |
|
| |
| [[de:Nephroblastom]]
| |
| [[fr:Tumeur de Wilms]]
| |
| [[es:Tumor de Wilms]]
| |
| [[hu:Wilms-tumor]]
| |
| [[nl:Nefroblastoom]]
| |
| [[pl:Nephroblastoma]]
| |
| [[pt:Tumor de Wilms]]
| |
| [[ru:Нефробластома]]
| |
| [[fi:Wilmsin kasvain]]
| |
|
| |
|
| |
|
| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}} | | {{WikiDoc Sources}} |