Etiology/ Pathophysiology of Wilm's Tumor

http://www.urologist.com.sg/urology-problem/kidney-cancer.html

What causes Wilm’s Tumor?

Similar to most cancers, the exact cause of Wilm’s Tumors is not known. In general, we know that cancer is a product of DNA mutations that cause cells to grow and divide abnormally. Many studies have been conducted trying to pin point the exact etiology of Wilm’s Tumor. Although no concrete evidence suggests a single cause or mutation in a child’s genetic make-up, some strong associations have been found. Researchers believe that the WT1 gene, which is located on the short arm of chromosome 11, may play a significant role in the development of Wilm’s Tumor. The WT1 gene plays an important role in the normal development of the kidney and urinary system. When the DNA that encodes for the WT1 gene experiences changes (also known as a mutation), the gene cannot function properly, and thus will be unable to carry out its given job in the body.

In addition to mutations in the WT1 gene, researchers have also considered the possibility that Wilm’s Tumor occurs as a result of a disruption of a gene called p53. The p53 gene is a special gene known as a “tumor suppressor” gene. It functions to remind cells to stop dividing and die when their work in the body is done. When the p53 gene is not working, cells are not reminded to stop, and as a result a tumor can develop.

Additionally, certain syndromes can also put a child at higher risk for Wilm’s Tumor. Children with WAGR syndrome (Wilms tumor-aniridia-genitourinary malformation-retardation), Denys-Drash syndrome, and Beckwith-Wiedemann syndrome are all considered to be at higher risk for the development of nephroblastoma.

For much more detailed and in depth analysis of the etiology/ pathophysiology of Wilm’s Tumor go to:

• http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3589819/ - article provides a comprehensive overview of Wilm’s Tumor
• http://emedicine.medscape.com/article/989398-overview#aw2aab6b2b3ab1 - website provides an overview of Wilm’s tumor etiology

What is the disease progression?

Wilm’s Tumor, as with most types of cancer, progress through a series of stages. In stage I the tumor is only in one kidney and is able to be completely removed by surgery. In stage II, the tumor has invaded near by tissues and blood vessels, however it can still be completely removed by surgery. In stage III, the tumor has spread beyond the kidney into the abdominal space. In stage III all of the cancer cannot be removed through surgery. In stage IV, the cancer has spread to distant areas of the body such as the brain, lungs, and bones. The later the stage, the farther cancer has spread. As cancer spreads to distant areas of the body, the function of those distant organs are compromised, accounting for the increasing morbidity and mortality as the progression continues. 

Sources:

Davidoff, A. M., & Advances in Pediatrics. (January 01, 2012). Wilms Tumor. Advances in Pediatrics, 59, 1, 247-267.

Disease Information: Solid Tumor: Wilms Tumor. (2015, January 1). Retrieved January 23, 2015. http://www.stjude.org/wilms-tumor

How is Wilms tumor staged? (2014, February 14). Retrieved January 23, 2015, from http://www.cancer.org/cancer/wilmstumor/detailedguide/wilms-tumor-staging

Paulino, A. (2015). Wilms Tumor . Retrieved January 23, 2015, from http://emedicine.medscape.com/article/989398-overview#aw2aab6b2b3ab1