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wilms' tumour
| Wilms' tumour is also known as nephroblastoma, as it arises in the kidney. Almost 90% of all kidney cancers are rare forms of childhood kidney cancers: clear cell sarcoma of the kidney, and occasionally renal cell carcinoma. Wilms' tumour is the second most common type of all childhood solid tumours, not including brain tumours. It can occur in children of all ages, but most are under five years of age. The tumour usually develops in only one kidney. Rarely it occurs in both kidneys.
Wilms' tumour is believed to result from mutations or change in certain genes. The involved genes and othe genes located nearby are only associated with Wilms tumour, but several other rare conditions. Wilms' tumour is strongly associated with several congenital abnormalities, notably anaridia (a rare condition, in which there is incomplete formation of the iris of the eye, can lead to blindness) and with some specific syndromes such as Beckwith-Wiedemann syndrome.
| symptoms
| Many children with Wilms' tumour have no symptoms at all and may be diagnosed when the child is evaluated for other reasons. Often a parent will notice a lump or mass in the child's abdomen, or the child may complain of abdominal pain. Sometimes the child may pass some blood in his/her urine.
| tests
| An ultrasound examination and a CT scan of the abdomen will be done to show the position of the tumour in the abdomen. A CT scan of the chest will also be done to find out if the cancer has spread to other parts of the body. This is known a staging, for example, Stage I refers to a tumour which is limited to the kidney and is able to be completely removed surgically. Other tests may be necessary, including blood tests to check the liver and kidney functions.
| treatment
| Treatment may be immediate surgery to remove the kidney and the tumour. It is perfectly possible to live with only one kidney. Alternatively, chemotherapy may be given first to shrink the tumour so that the operation is easier.
At the operation the surgeon will examine the whole abdomen and will take specimines of lymph glands, to determine if they are affected. The best treatment for each child with Wilms' tumour is dependent on told by a pathologist. Depending on the type of cells seen, the tumour is said to be either of favourable or unfavourable histology. The vas majority, approximately 95% of children diagnosed with Wilms' have cells with a favourable histology.
Treatment is dependent on the extent of the tumour. Chemotherapy may be given for up to one year at regular intervals. Radiotherapy may also be needed.
| Treatment of Wilms' tumour in children is one of medicines success stories. Due to improvements in surgical techniques, chemotherapy drugs and radiation, 90% of children with Wilms' tumours will be cured.
| follow up care
| Most children recover quickly from the operation. Follow up care involves regular clinical examinations, abdominal ultrasound's and chest x-rays.
After the first few years the focus of follow up changes to monitoring growth and development and other possible later side effects of treatment.
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