Wilms' tumor pathophysiology: Difference between revisions

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Revision as of 13:44, 17 September 2012

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pathophysiology

Pathologically, a triphasic nephroblastoma comprises three elements:

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.

The mesenchymal component may include cells showing rhabdomyoid differentiation. The rhabdomyoid component may itself show features of malignancy (rhabdomyosarcomatous Wilms).

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)

@@ Line 18: / Line 18: @@
 * ''Favorable'' - Contains well developed components mentioned above
 * ''[[Anaplastic]]'' - Contains diffuse anaplasia (poorly developed cells)
-===Molecular biology===
-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>
-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>
 ==References==
 {{Reflist|2}}

Wilms' tumor pathophysiology: Difference between revisions

Revision as of 13:44, 17 September 2012

Overview

Pathophysiology

References

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