Wilm's Tumor

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[edit] Name of Condition

Wilm's Tumor, also known as Nephroblastoma.

[edit] Definition

Wilm's Tumor is a type of cancer that occurs in the kidneys of children, and is the most common type of childhood kidney cancer. The cancer can occur in one or both kidneys. The average age of diagnosis is 3.5 years, and it is rarely seen after age 8. Wilm's Tumor develops when some immature cells in the child's developing kidneys begin to grow out of control, forming a tumor. Thanks to research and advancements in the last few decades, there are many options to help children who develop Wilm's Tumors, and the survival rate is good.

[edit] Description, Signs and Symptoms

Some symptoms of Wilm's Tumor are:

  • An abdominal mass
  • Fever
  • Blood in the urine
  • Reduced appetite
  • Weight loss
  • High blood pressure
  • Constipation
  • Stomach pain
  • Nausea
  • Vomiting
  • General discomfort (malaise)

[edit] Inheritance

Only about 5% of cases of Wilm's Tumor are inherited, while most are sporadic. The tumor originates when tumor suppressor genes fail to function properly. Two mutations can cause this loss of function. On the tumor suppressor gene, the deactivation of the first allele has two effects. One effect can take place in all cells of the body, and can predispose the patient to inherited or multiple Wilms Tumor. The other effect will only take place in specific cells, which would cause sporadic or single tumors. The deactivation of the second allele on the tumor suppressor gene may also have effects on Wilm's Tumor, but these causes are still being investigated.

[edit] Demographics

Wilm's Tumor is estimated to affect 1 in 10,000 children under 14.

[edit] Diagnosis

Doctors can diagnose Wilm's Tumor upon observation of the symptoms and by doing an ultrasound, CT scan, or an MRI to determine if there is cancer in the kidneys. If there is a tumor, the doctor may do an X-ray, chest CT scan, chest MRI, or a bone scan to determine if the cancer has spread to other parts of the body.

[edit] Treatment and Management

The most common treatment for Wilm's Tumor is surgery to remove the tumor from the kidney(s), followed by chemotherapy. Radiation may be used, depending on the type of cells that are observed.

[edit] Prognosis

Today, most children who have their tumor detected early can undergo surgery, and in 85% of cases it is effective and allows the child to go on and live a complete, normal life. In some cases, the tumor is not detected until it has metastasized, it may be fatal for the child, or if the condition is complicated by other genetic conditions.

[edit] Resources

http://www.nwtsg.org/

http://www.kidney.ca/page.asp?intNodeId=22145

[edit] References

http://www.emedicine.com/med/topic3093.htm

http://www.cancer.gov/cancertopics/pdq/treatment/wilms/Patient

http://www.acor.org/ped-onc/diseases/wilms.html

http://www.mayoclinic.com/health/wilms-tumor/DS00436

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