Wilms' tumor pathophysiology: Difference between revisions
Shanshan Cen (talk | contribs) No edit summary |
Shanshan Cen (talk | contribs) |
||
| Line 51: | Line 51: | ||
==Genetics== | ==Genetics== | ||
Increasingly gene loci are being implicated on chromosome 11 (WT1: 11p13 and WT2: 11p15) as well as other loci on chromosomes 1, 8 and 12. <ref name=radio> Wilms tumour. Dr Tim Luijkx and Dr Frank Gaillard et al. Radiopaedia.org 2015.http://radiopaedia.org/articles/wilms-tumour </ref> | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
Revision as of 18:32, 25 August 2015
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
|
Wilms' tumor Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Wilms' tumor pathophysiology On the Web |
|
American Roentgen Ray Society Images of Wilms' tumor pathophysiology |
|
Risk calculators and risk factors for Wilms' tumor pathophysiology |
Overview
The tumour typically arises from mesodermal precursors of the renal parenchyma (metanephros). Increasingly gene loci are being implicated on chromosome 11 as well as other loci on chromosomes 1, 8 and 12.
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.
Wilms’ tumor is thought to arise from pre-existing lesions known as nephrogenic rests. In unilateral tumors, nephrogenic rests might be a prognostic factor for recurrence in the opposite kidney. Nephrogenic rests may be hyperplastic (masses) or schlerotic (fibrous) and may have immature tubular forms.
They commonly have a capsule and are vascular. They rarely cross the midline. Metastases are most often to the lungs.
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)
Shown below is a pathology image depicting a cut section showing two halves of a nephroblastoma specimen. The image is courtesy of Chimene Kesserwan.
Shown below is a series of microscopy image showing the characteristic triphasic pattern consisting of tubules, solid sheets of small round cells, and stroma on H&E stain. The images show the characteristic three components:
- Malignant small round (blue) cells ~ 2x the size of resting lymphocyte (blastema component).
- Tubular structures/rosettes (epithelial component).
- Loose paucicellular stroma with spindle cells (stromal component).
 |
|
| Low magnitude | Intermediate magnitude |
 |
|
| High magnitude | Very high magnitude |
Genetics
Increasingly gene loci are being implicated on chromosome 11 (WT1: 11p13 and WT2: 11p15) as well as other loci on chromosomes 1, 8 and 12. [1]
References
- ↑ Wilms tumour. Dr Tim Luijkx and Dr Frank Gaillard et al. Radiopaedia.org 2015.http://radiopaedia.org/articles/wilms-tumour