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WILMS TUMOR

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WILMS TUMOR

Wilms’ tumor is a type of rare childhood cancer that starts in the kidney.

  • Wilms’ tumor also known as nephroblastoma is the most common form of kidney cancer in children and the fourth most common type of childhood cancer.
  • Wilms tumor is prevalent in children ages 4-5 years and becomes much less common after age 5.
  • Most Wilms’ tumor is unilateral, meaning they affect only one kidney.
  • They are bilateral, found in both kidneys, in about 5% to 10% of children with the condition.
  • Wilms’ tumor can metastasize to the lungs, it may also put the patient at risk of bleeding and peritoneal dissemination of the tumor if ruptured.

CAUSES

The exact cause of the condition is unknown. Researchers believe that certain genetic factors may play a role.

Researchers believe that alteration or mutations in Wilms Tumor 1 (WT1) or Wilms’ Tumor 2 (WT2) gene plays a role. These genes are located on chromosome 11 and help in suppressing tumors. Changes in these genes account for a small percentage of the disease. Frasier syndrome, WAGR syndrome, and Denys Dash syndrome are genetic syndromes that may increase a child risk for Wilms tumor.  They are linked to mutations in the WT1 gene.

Beckwith-Weidman syndrome is also a genetic syndrome that may increase a child risk for Wilms tumor. But it is linked to mutations in WT2.

Other genetic syndromes that prone as risk factors are:

  • Perlman syndrome
  • Sotos syndrome
  • Bloom syndrome
  • Trisomy 18, also called Edwards syndrome
  • Simpson-Golabi- Behmel syndrome

It is also believed that fragments of tissues (nephrogenic rests) in or around the kidney which develops before birth may become cancerous after birth. Many, but not all cases of the disease develop from nephrogenic rests. Nephrogenic rests are precursors of Wilms’ tumor.

Children with birth defects such as aniridia and hemihypertrophy are more likely to have the disease.

African-American children are more likely to develop the disease than children of other races.

A family history of the condition may also be a risk factor.

SYMPTOMS

Symptoms may vary widely, and some children don’t show obvious symptoms. If symptoms exist, they may include:

  • Abdominal pain
  • Abdominal swelling
  • A painless abnormal mass that can be felt
  • Fever
  • Blood in the urine or discoloration of urine
  • Loss of appetite
  • Weakness and fatigue
  • Nausea and vomiting
  • High blood pressure
  • Constipation
  • Unexplained weight loss

DIAGNOSIS AND TREATMENT

Over the years, advancements in the diagnosis and treatment of condition have greatly improved the outlook for children with the disease.

With appropriate treatment, the outlook is very good.

To make a diagnosis, the doctor will carry out a physical examination to look for possible signs of the condition.

Blood and urine tests to detect Wilms’ tumor, indicate how well the kidneys are working as well as detect certain kidney problems.

Imaging tests such as CT scan, ultrasound, and MRI scans can also be used.

Once the condition is diagnosed, the doctor will determine the extent of cancer either by carrying out a chest X-ray or chest CT scan and bone scan.

The stages range from   I-IV

Stage 1, the cancer is confined to the kidney and can be completely removed by surgery.

Stage 2, cancer has spread to the tissues and structures (such as nearby fats and blood vessels) beyond the affected kidney but can still be completely removed by surgery.

Stage 3, cancer has spread beyond the kidney area to nearby lymph nodes or other structures within the abdomen, and cannot be completely removed by surgery.

Stage 4, cancer has spread to other structures such as the lungs, brain, liver, or bone.

Stage 5, the cancer is in both kidneys (bilateral).

TREATMENT

Treatment option may include:

Surgery to remove part of the affected kidney (partial nephrectomy), to remove the affected kidney and surrounding tissues (radical nephrectomy), or to remove all or part of both kidneys.

Chemotherapy that involves using medication injected intravenously or taken orally to kill the cancer cell.

Radiation therapy that uses powerful, energy beams such as X-rays to kill the cancer cell

Clinical trials which are research studies that test out new treatment or procedures.

According to the American Cancer Society, doctors recommend that children with a higher risk of the condition get an exam every three to four months until they reach 8 years.