Wilms Tumour Overview: Symptoms, Risk Factors, and Treatments

Wilms tumour is one of the first cancers to be seen in children, and it is most commonly diagnosed in kids. It affects nearly 1 in 10,000 children, and each year, the United States sees about 500 to 600 new cases diagnosed.

In order to diagnose and treat such a tumour, early recognition of Wilms tumour symptoms and risk factors is crucial.

Thus, discussing the pathophysiology, causes, stages, and treatment options for Wilms tumour can help familiarize people with the process. Preventive measures and early detection are essential to improving outcomes so that such children receive the care needed to combat this disease effectively.

Keep reading to know more in-depth details about Wilms tumour symptoms and treatments.

What is Wilms Tumour?

Wilms tumour definition is basically that it is a rare kidney cancer that preferably hurts children, who are usually 3 to 4 years of age, and more than 80% of children develop the tumour before attaining age 5. The tumour primarily occurs in one kidney, but in some cases, it can be found in both kidneys.

Often, Wilms tumour is linked to conditions that exist at birth, known as congenital syndromes. This disease is relatively less common among Asian children.

The Wilms tumour symptoms may include abdominal pain and swelling or even blood in the urine or high blood pressure, which may prompt parents to call their paediatrician.

Types of Wilms Tumours

Based on the appearance of the cells at the microscopic level, Wilms tumours in children can be divided into two groups. The types of Wilms tumour are discussed below in the following table:

Types Description

Favourably Histological This category possesses over 90% of the Wilms tumours and shows very few differences among the tumour cells. Most of the children with this variant are expected to be treated successfully.

Unfavourable or Anaplastic Histology It comprises a spectrum of abnormal cancer cell formations, making treatment incredibly difficult.

Pathophysiology of Wilms Tumour

Wilms tumour pathophysiology has been associated with the development of abnormal kidney cells that grow without control. These cells typically stem from immature kidney cells, known as nephroblasts, which do not develop as they should during fetal development. As a result of their abnormal growth, these cells form a tumour inside the kidney.

Causes of Wilms Tumour

The exact causes of Wilms tumour are still unknown. Cancer occurs when body cells grow in an uncontrolled manner. If a child has a Wilms tumour, it means their kidney cells did not develop properly and became cancerous. 
Commonly, this is caused by a random gene change. Sometimes, it may result from inherited gene changes passed down from one of the parents. There are certain risk factors that you need to see, which can lead to Wilms tumour in young children.

Risk Factors of Wilms Tumour

Here are the major risk factors that can often cause Wilms tumours in young ones:

Genetic and Familial Associations

Although it is not known why people develop Wilms tumours, genes have a primary role. Several genetic syndromes or disorders increase the risk of developing a Wilms tumour. These include Beckwith-Wiedemann syndrome, Denys-Drash syndrome, and WAGR syndrome: aniridia, genitourinary anomalies, and intellectual disabilities. 

A family history of Wilms tumour is another significant risk factor, as genetic mutations and abnormalities in the tumour-suppressor genes are often implicated in the causes of Wilms tumour.

Environmental Factors

Although genetic issues are significant, environmental factors may contribute to the development of Wilms tumour. They have been proven to alter a baby's risk of developing this disease when exposed to any chemicals or radiation during pregnancy. Besides, pregnant women are recommended to avoid harmful agents as much as possible during their pregnancy period to reduce the hazard.

Age and Sex

Wilms tumour is diagnosed most frequently in children under the age of 5, peaking between the ages of 3 and 4. It has a slightly increased incidence in girls as compared to boys. Age and gender distribution are critical factors when looking into the symptoms and possible diagnosis of Wilms tumour because, typically, most of these children are diagnosed in early childhood.

Background and Risk Variation

Some studies have determined that there is a relatively higher rate of Wilms tumours observed among children from African and Caribbean backgrounds in North America and Europe. This may be due to genetic factors unique to these populations. 

Children of Asian descent have shown a lower rate of the disease, and thus, genetic, or environmental factors are speculated to be specific to this group and to be related to the reduced risk of developing the condition. 
Understanding these ethnic variations can help provide ample information for identifying high-risk groups and tailoring early detection approaches for effective intervention.

Symptoms of Wilms Tumour

Mentioned below are the common Wilms tumour symptoms in kids:

• Belly pain
• Swelling in their belly
•  Fever 
• A growth that you can see or feel in their belly.
• High blood pressure
• A swollen area or hard bump in your child's belly.
• Nausea
• Lack of appetite
• Shortness of breath
• Blood in urine (hematuria)
• Constipation

Stages of Wilms Tumour

The stages of Wilms tumour signify how widely the tumour grows within the kidney or into other parts of the body. Here are the five main stages of Wilms tumour:

Stage 1: The tumour can be completely removed by surgery since it's only in the kidney and has not spread to nearby tissues or distant organs.
Stage 2: The tumour has invaded adjacent tissues or blood vessels but remains confined to the kidney region.
Stage 3: In this stage, the tumour extends to the surrounding lymph nodes or organs in the abdominal chamber.
Stage 4: The tumour extends to distant organs such as the lungs, liver, or bones.
Stage 5: There have been tumours in two kidneys.

Wilms Tumor Diagnosis

If you see a lump in your baby's diaper area, you should immediately consult a doctor about Wilms tumour diagnosis. These tumours can be quite large, sometimes even bigger than the kidney. If your child suffers from a syndrome or genetic condition associated with Wilms tumour, then the doctor might suggest a routine test for it.

• A physical examination in which the doctor will gently press the child's abdomen.
• Imaging tests, such as an abdominal ultrasound or CT scan, often with contrast. Your doctor may order an X-ray or CT scan of the chest to see if the cancer has spread to the lungs. These imaging tests help identify the tumour and distinguish Wilms tumour from other kidney cancers.
• Blood and urine tests, including checks for liver function and blood clotting.
• A biopsy, where an example of tumour tissue is taken for lab tests.

Wilms Tumor Treatment

The extent of the spread of cancer determines the approach of Wilms tumour treatment. Surgery, chemotherapy, and radiation therapy may all be used. Here are a few of them:

Surgery

If surgical intervention is essential, the following methods may be performed:

• Partial Nephrectomy: In this procedure, the tumour, together with a margin of healthy tissue surrounding it, is removed.
• Radical Nephrectomy: This is a surgical procedure in which the entire diseased kidney, the ureter (the duct that carries urine from the kidney), the adrenal gland resting on top of the kidney, and all adjacent tissues are removed
• Bilateral Nephrectomy: In some instances, the child will require both kidneys to be removed. In such cases, the child will have to undergo dialysis to remove waste products from their bloodstream. Subsequently, after their health improves, a kidney transplant would be recommended.

Chemotherapy 

Almost all children with Wilms tumours undergo chemotherapy at some point in their treatment. However, this treatment can be pretty effective but will also impact the normal cells in the child and exhibit effects with symptoms like hair loss, tiredness, sores in the mouth, nausea and vomiting, and changes in bowel habits, among others.

Radiation

Radiation therapy is one of the most efficient ways of targeting and killing cancer cells. A dedicated machine perfectly places the radiation onto the tumour. Oncologists frequently administer radiation therapy in stages from stage III and beyond.

Briefly, Wilms tumour is an aggressive kidney cancer that mainly affects children. Timely detection of Wilms tumour symptoms is crucial in enhancing the survival rate. Risk factors, pathophysiology, and treatment options related to this subject matter have improved families' lives with the number of challenges associated with this disease.

Although preventive measures are helpful, early screening and treatment can improve the quality of life. For your family's safety and well-being, consider investing in health insurance from Star Health Insurance that ensures comprehensive health coverage of pediatric care as well as treatment for peace of mind during difficult times.