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Nephroblastoma, also known as Wilms tumor, is the most common kidney cancer in children. It has an incidence rate of 0.8 cases per 100,000 people and is typically diagnosed in children between the ages of three and four. The success rate for treating Wilms’ tumor is around 90% due to a combination of surgery and radiation or chemotherapy. Symptoms include abdominal pain, a lump in the abdomen, fever, anemia, and blood in the urine. Treatment involves surgery to remove the tumor or kidney followed by chemotherapy.
Nephroblastoma is a type of cancer that develops in the kidneys. This cancer is also called Wilms tumor and is the most common type of kidney cancer to develop in children. In the 1950s and 1960s, most children with nephroblastoma had a very poor prognosis, but treatment for the cancer advanced significantly in the decades that followed. The success rate for treating Wilms’ tumor is now around 90%, largely due to the practice of combining surgery with radiation or chemotherapy.
Nephroblastoma has an overall incidence rate of 0.8 cases per 100,000 people. Most children with this cancer are diagnosed between the ages of three and four. Children commonly have tumors in only one kidney; in about 6% of cases, the tumors grow in both kidneys. Typically the development of cancer is spontaneous; however, it can sometimes arise as part of a syndrome such as congenital aniridia, in which the risk of Wilms’ tumor is linked to eye disorders such as cataracts and glaucoma. Susceptibility to this type of cancer can also be genetically inherited, although increased susceptibility does not guarantee that any given individual will develop cancer.
Many types of cancer are caused by mutations in tumor suppressor genes. These are genes that, when functioning properly, reduce the risk of cells becoming cancerous. Many different genes, with a wide range of functions, can act as tumor suppressor genes. The gene that is linked to nephroblastoma is active during fetal kidney development but is relatively inactive after birth. A mutation in this gene promotes cellular changes that can cause a Wilms tumor to develop.
The most common symptoms of nephroblastoma are abdominal pain and a lump in the abdomen. Many children also have fever, anemia, or blood in their urine. Possible complications include metastases, which commonly occur in one lung. Additionally, a Wilms tumor is at risk of rupturing, which can lead to kidney hemorrhage and the shedding of cancer cells throughout the abdomen.
Children diagnosed with Wilms’ tumor usually have surgery to remove the tumor or, if necessary, a whole kidney. This surgery is followed by chemotherapy to ensure that all cancer cells are killed. In rare cases, radiation therapy may be used as a follow-up treatment instead. Generally, however, the established protocol for treating this cancer is surgery and chemotherapy with a specific set of drugs. The most commonly used chemotherapeutic agents to treat cancer are doxorubicin, vincristine and dactinomycin®.
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