Wilms Tumour - Medical Malpractice Lawyers

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Wilms Tumour - Medical Malpractice

Wilms tumour is also called nephroblastoma and represents a type of cancer of the kidneys that usually affects children, although adults can rarely be affected. It was named after Dr. Max Wilms who lived in the latter half of the 1800s and was the first to identify this particular type of kidney tumour. Wilms tumour is an uncommon tumor occurring in about 500 children in the US each year. Most develop in normal kids who happen to get cancer. About 25 percent of cases develop in children who have some other developmental abnormality. Most patients survive the treatment of Wilms tumour-about 90 percent are still alive after five years post-diagnosis. Most cases of Wilms tumour occur on only one side of the body; however, 5 percent are bilateral.

There are two types of Wilms tumour. The first is the favourable type with well developed histopathological components and slow growth. The second is the anaplastic type, which contains rapidly growing, poorly developed cells and a much worse prognosis than the favourable type. Some people with Wilms tumour have a genetic abnormality on chromosomes 1p and 16q. These patients have a higher risk of getting Wilms tumour and a greater risk of dying from the disease. There are other genetic mutations within Wilms tumours that are the cause of the tumour occurring.

Wilms tumour has several stages that define how it is treated. Forty three percent of cases are found at stage I. This means that the tumour is located within the kidney and can be completely removed surgically. The capsule of the kidney is completely intact and the tumour hasn't been biopsied via a needle or open biopsy before its removal. There are no lymph nodes involved. The treatment of this stage of the disease is a total nephrectomy (removal of the kidney) and chemotherapy for around 4 to 5 months. If the child is less than two years old and the tumour weighs less than 550 grams, only surgery is necessary. Ninety eight percent of these people survive to four years.

Stage II disease involves 23 percent of patients. The tumour extends beyond the boundaries of the kidney but can be completely removed. There is no residual tumour left and no involvement of blood vessels near the kidneys. If the tumour has been biopsied in the past, it is considered a stage II tumour. The treatment for this type of cancer is removal of the kidney along with radiation to the abdomen and 24 weeks of extensive chemotherapy. The outcome is good with 96 percent of cancer patients surviving past four years unless the disease is anaplastic. Then only 70 percent of patients survive past 4 years.

Stage III Wilms tumour occurs in 23 percent of cases. In such cases, the tumour cannot be resected entirely and there are metastases to local lymph nodes. Tumour is left behind by necessity or has been spilled onto the peritoneum during surgery. The treatment is radiation to the abdomen and chemotherapy for 24 weeks. The nephrectomy is performed after the cancer has shrunk somewhat. The survival rate is 95 percent after 4 years or 56 percent, if the tumour is considered anaplastic.

Only about 10 percent of patients are found in stage IV. This involves metastases to other body areas, usually via the blood stream. Common places for metastases include lung, bone, liver and brain and there are lymph nodes involved outside of the region of the abdomen and pelvis. The treatment is to remove the kidney and its tumour, provide abdominal radiation and give 24 weeks of chemotherapy. Sometimes large metastases are treated by radiation as well. The survival rate is 90 percent in favourable tumours and only 17 percent in anaplastic tumours.

There is such a thing as stage V Wilms tumour. This is when both kidneys are involved at the time of first diagnosis. Each side is staged separately as well. The four year survival rate is about 94 percent for those who have stage I or II on both sides and 76 percent if the most advanced lesion was found to be a stage III or more.

Anaplastic tumours do not mean automatic death. The vast majority survive to reach five years post diagnosis but tend to be treated more aggressively than favourable Wilms tumour.

The author of the substantive medical writing on this website is Dr. Christine Traxler MD whose biography can be read here