Wilm's tumor is a rare kind of kidney cancer that occurs in children. It can occur in one or both kidneys. Around 500 children are newly diagnosed every year, and it is the fourth most common type of cancer among children. Wilm's tumor is also known as nephroblastoma. It is often noticed because of abdominal swelling or any mass in the kidney. Some patients with Wilms' tumors have congenital disabilities such as urinary tract abnormalities or lack of an iris in the eye.

What Are the Types of Wilm's Tumor?

Favorable Histology: The cancer cells in these tumors do not look normal, but there is no anaplasia. About 9 of 10 Wilm's tumors have a favorable histology. The chances of curing children with these tumors are excellent.
Anaplastic Histology: The cancer cells may vary widely in shape and size in these tumors. The nucleus of the cells (where the DNA is present) tends to be very big and altered, called anaplasia. The tumors in which the anaplasia spread throughout the tumor, known as diffuse anaplasia, are more challenging to treat. The anaplasia is limited to certain parts of cancer, known as focal anaplasia.

What are the Symptoms of Wilm's Tumor?

The symptoms of Wilm's tumor include:

An abdominal mass.
Abdominal pain.
Abdominal swelling.

The other signs and symptoms may include:

Nausea or vomiting or both.
Fever.
Constipation.
Blood in the urine.
Loss of appetite.
Shortness of breath.
High blood pressure.
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What Causes a Wilm's Tumor?

1. Genetic Factors

The following genetic factors might increase the risk of the child getting Wilm's tumor.

Beckwith-Wiedemann syndrome.
WAGR (Wilms tumour-aniridia) syndrome.
Perlman syndrome.
Frasier syndrome.
Denys-Drash syndrome.
Trisomy 18 or Edward's Syndrome.
Li-Fraumeni syndrome.
Sotos syndrome.
Simpson-Golabi-Behmel syndrome.
Bloom syndrome.

2. Race - Wilm's tumor is more commonly seen in African- American children, and it is less common in Asian-American children.

3. Gender - The risk is higher in females than males.

What Is the Pathology Behind Wilm's Tumor?

Wilms' tumor occurs due to alterations to genes such as the Wilm's tumor 1 (WT1) or Wilms Tumor 2 (WT2) genes. The kidney cells usually develop in the womb as the fetus grows, but some of the cells which fail to develop into mature cells remain the same. Usually, these cells will mature before the child reach three to four years of age. When this does not occur, the cells might grow out of control and result in Wilm's tumor.

Who Are at Risk for Wilm's Tumor?

Wilm's tumor is most common in young children who are around three to four years of age and less common in older children. And if any of the child's family members have had Wilm's tumor, then that child has an increased risk of acquiring the condition.

How to Diagnose and Stage Wilm's Tumor?

Wilm's tumor is diagnosed before the age of ten.

1. An ultrasound can be done first to confirm the presence of intrarenal mass.

2. CT (computed tomography).

3. MRI (magnetic resonance imaging).

4. Biopsy.

5. X-rays to look for metastasized areas, usually in the lungs.

6. Radioactive materials are used in smaller quantities to highlight the areas of diseased bone.

7. Laboratory tests like blood tests and urine tests are done to check a child's general health and detect any adverse side effects (like low red or white blood cell counts) of the treatment.

Doctors usually use a staging system to find the extent of cancer. There are about five stages:

Stage 1: The tumor is present in one kidney and can be removed entirely with surgery. There are about 40 to 45 % of stage 1 Wilms' tumors.

Stage 2: The tumor spreads into the tissue and vessels around the kidney, but still, it can be completely removed with surgery. About 20% of Wilms' tumors are stage 2.

Stage 3: In this, the tumor cannot be completely removed with surgery, and some cancer remains in the abdomen. About 20 to 25% of Wilms' tumors are stage 3.

Stage 4: In this, cancer has spread to distant organs, such as the lungs, brain, or liver. About 10% of Wilms' tumors are stage 4.

Stage 5: The tumor is present in both kidneys at the time of diagnosis. About 5% of Wilms' tumors are stage 5.

How to Treat or Manage Wilm's Tumor?

Most of Wilm's tumors are of favorable histology, and they are easy to cure.

Surgery is the usual treatment for Wilms tumor. In a radical nephrectomy, the surgeon removes:

The tumor.
The entire kidney.
Ureter.
Adrenal gland (a gland that is on top of the kidney).
Surrounding fatty tissues.
Nearby lymph nodes.

When cancer is present in both the kidneys, surgeons will usually remove the tumor and preserve the healthy kidney tissue to avoid kidney transplants. Treatment plans include both chemotherapy and surgery. More advanced stages also may require radiotherapy. Both treatments have short-term and long-term risks.

Short-term or temporary effects may include:

Nausea and vomiting.
Bleeding or bruising.
Loss of appetite.
Tiredness.
A weakened immune system.
Nephrectomy.
Mouth sores.
Hair loss.

Long-term effects may include:

The development of secondary cancers (such as leukemia).
The weakening of any internal organs, such as the heart.

What Is the Prognosis of Wilm's Tumor?

With proper treatment, children with Wilm's tumor have a 90 percent survival rate. However, the chances of recurrence are between 15 to 50%, depending on the original tumor's traits. The people who had Wilm's tumor may experience related health problems or late effects of their treatment in adulthood, such as heart disease, decreased kidney function, and additional cancer development.

How to Prevent Wilm's Tumor?

There is no prevention for Wilm's tumor, but the kids at risk for Wilms tumor should undergo an ultrasound screening every three months until about six or seven years of age. Those at high risk may get screened until they are a little older. For more information, consult a doctor online at iCliniq.com.