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Wilms Tumour: Symptoms, causes, prevention and treatment

Dr. Rashmi ByakodiJan 17, 2024

Cancer occurs when abnormal cells invade your healthy cells and multiply uncontrollably. It can attack people at any age, including childhood. Some cancers exclusively occur in childhood; Wilms Tumour is one of them. Read ahead to know more about this disease and its symptoms, causes, prevention methods, and treatments.

Contents

What is Wilms Tumour?
Causes of Wilms Tumour
Symptoms of Wilms Tumour
Types of Wilms Tumour
Diagnosing Wilms Tumour
Wilms Tumour’s stages
Treatments of Wilms Tumour
Wilms Tumour prognosis
How to prevent Wilms Tumour
Frequently Asked Questions (FAQs)

What is Wilms Tumour?

Also known as Nephroblastoma, Wilms Tumour is a kind of Kidney Cancer that predominantly affects kids under the age of five. It generally affects only one kidney. However, the American Cancer Society indicates about 5% to 10% of children may develop tumours in both kidneys. Wilms Tumours usually become much larger than the kidney from which they started. But the good part is that they often get detected before metastasising.

Causes of Wilms Tumour

Though the exact cause of Wilms Tumour is not known, there are a few possible risk factors that researchers have identified. They may include the following.

1. Genetics

Researchers consider that certain genetic factors might increase a child's risk of Wilms Tumour. This could be because Wilms Tumour often mutates with certain genes like the WT1 gene, the CTNNB1 gene, or the AMER1 gene. These three genes help the body manage cell growth and make proteins.

Apart from the above genes, the following genetic syndromes may also raise the risk of Wilms tumour in a child.

Beckwith-Wiedemann Syndrome: It's a rare genetic condition where body organs grow larger than usual. Children with Beckwith-Wiedemann syndrome have a 5% to 10% chance of developing Wilms Tumour.
WAGR syndrome (Wilms Tumour, aniridia, genitourinary anomalies, and intellectual disability): WAGR syndrome is a rare genetic disease characterised by a wide range of disorders, including distinctive eye disorder and hypospadias in males, urinary abnormalities in females, certain cancers, intellectual disability, and developmental delays. Children born with WAGR syndrome may have a 50-50 chance of developing Wilms Tumour.
Denys-Drash Syndrome: This is an abnormal kidney condition that begins at the stage of infancy and usually leads to permanent kidney failure. Children with Denys-Drash syndrome have a 90% chance of developing Wilms Tumour.

Sometimes, Wilms Tumour is associated with other syndromes as well, such as Frasier Syndrome, Simpson-Golabi-Behmel Syndrome, Bloom Syndrome, Perlman Syndrome, Sotos Syndrome, Li-Fraumeni syndrome, and Trisomy 18.

Children having some developmental anomalies, as mentioned below, are more likely to have Wilms Tumour.

Aniridia: A condition where a child has a total or partial missing of the iris.
Hemihypertrophy: One part of the body is bigger than the other.
Cryptorchidism: The testicles are undescended.
Hypospadias: A condition where the urinary opening is underneath the penis.

2. Race

As per the American Cancer Society, Wilms Tumour affects black children marginally more often than white children.

3. Gender

Girls are believed to be more vulnerable to this disease than boys.

Symptoms of Wilms Tumour

The most common symptom of Wilms' Tumour is swelling in the abdomen. This is usually painless, but it can be accompanied by a lump that the parent or carer can feel. It may also cause small amounts of bleeding in the abdominal area, which can irritate the kidney and cause pain.

Children with Wilms Tumour may also experience the symptoms mentioned below.

Vomiting or feeling of nausea
Constipation
Loss of appetite
Fatigue and weakness
Fever
Discoloured urine
Blood in urine
High blood pressure
Shortness of breath
Chest pain
Headaches

Types of Wilms Tumour

Wilms Tumour can be categorised into two types on the basis of how the tumour cells look under a microscope.

1. Favourable Histology: It refers to a type of tumour that has significantly less anaplasia, meaning that the appearance of the cells slightly differs from normal cells. This is unlike a malignant tumour. This also means most of these tumours are easily curable.

2. Unfavourable Histology: It refers to a cancerous tumour that has a lot of anaplasia, meaning the appearance of cells is different from normal cells, and the nucleus looks abnormal, like in a malignant one. This makes it difficult to cure.

Diagnosing Wilms Tumour

The diagnosis process of Wilms Tumour may include the following steps.

Physical examination: Children who were born with any birth problems linked to Wilms' Tumour should be screened regularly for the disease with physical exams.
Blood test: Blood tests to look for anaemia and coagulation rates are done regularly in children at high risk for this condition.
Urine test: Is done to look for renal function.
Imaging tests: Like ultrasound, X-ray, CT scan, and MRI scan, PET/CT scan is used to detect the tumour and its location. It also helps in determining whether the cancer cells have spread to other parts of the body.
Biopsy: Doctors advise biopsy to know whether the tumour is favourable or anaplastic. Here, a small piece of the tumour is taken and evaluated under a microscope.

Wilms Tumour’s stages

The stages of Wilms Tumour are determined by examining how far it has spread. There are five stages of Wilms Tumour.

Stage 1: The cancer is confined within one kidney and is completely removable with surgery. About 40% to 50% of tumours are at stage 1.

Stage 2: Cancer has spread to the nearby tissues and vessels of the kidney but can still be completely removable with surgery. About 20% of tumours are at stage 2.

Stage 3: Cancer has spread to the nearby lymph nodes but not to the farther nodes. Some cancer may remain in the abdomen, and some may spread into nearby vital organs, so the tumour cannot be entirely removed. Seen in 20% to 25% of tumours.

Stage 4: Cancer has metastasised to distant organs such as the liver, lungs, brain, or bones. About 10% of tumours are at stage 4.

Stage 5: Cancerous cells are present in both the kidneys at the time of diagnosis. About 5% of tumours are at stage 5.

Treatments of Wilms Tumour

Children with Wilms’ Tumours are usually treated by a team of doctors, including paediatricians, surgeons, and oncologists.

1. Surgery

Surgery for Wilms Tumour can be partial nephrectomy, radical nephrectomy, or removal of both kidneys. In the first case, the tumour is removed along with some healthy tissues around it. Radical nephrectomy includes the removal of the affected kidney, nearby lymph nodes, and adrenal gland. Removal of both kidneys may be done if both kidneys are affected. In that case, the patient has to undergo regular dialysis until they become healthy enough for a kidney transplant.

2. Chemotherapy

During chemotherapy, certain powerful medications are injected intravenously to destroy cancer cells and restrict them from multiplying. However, these chemotherapy drugs kill healthy cells, which leads to several side effects such as loss of appetite, hair loss, nausea, vomiting, diarrhoea, etc.

3. Radiation Therapy

This method of treatment uses high-energy X-ray beams or other radiation variants to destroy the cancer cells or to stop them from multiplying.

Wilms Tumour prognosis

Most children with Wilms Tumour have a good prognosis. About 86% to 99% of children with favourable histology can be cured. However, patients with anaplastic histology have a lower rate of cure. Children need to see their doctor regularly after treatment so they can lead healthy lives.

How to prevent Wilms Tumour

Wilms Tumour occurs when certain cells in a child's kidneys fail to develop properly before birth. Wilms Tumour cannot be prevented, but doctors may be able to catch it early by monitoring a child's growth and development with regular physical exams. However, in rare cases like Denys-Drash Syndrome, the doctors may recommend removing the kidneys at a very early age to inhibit tumours from developing.

Children with genetic conditions and family history should visit the doctor regularly and get regular ultrasounds. This will help to detect Wilms Tumour early, and it can be treated quickly and effectively.

Frequently Asked Questions (FAQs)

Here are some common questions and their answers about Wilms tumours.

What is the best treatment for Wilms Tumour?

The best treatment for Wilms Tumour may include surgery, chemotherapy, and radiation therapy. However, these treatments may vary depending upon the stage of cancer. Surgery of Wilms Tumour may include the following.

Partial nephrectomy: removing the tumour along with some surrounding tissues
Radical nephrectomy: removing the affected kidney, the surrounding tissues, the ureter, the nearby lymph nodes, and the adrenal gland
Removal of both kidneys: removing both the affected kidneys and the patient has to be under dialysis

What are the risk factors for Wilms Tumour?

Some risk factors of Wilms Tumour may include certain genetic syndromes or birth anomalies, family history of Wilms Tumour, gender, age, and race.

How is Wilms Tumour diagnosed?

Besides the physical examination, the following tests may be done to diagnose Wilms tumour.

Blood and urine tests
X-ray
Ultrasound of abdomen
CT Scan
MRI Scan
Bone Scan
Biopsy

What is the survival rate of Wilms Tumour?

The 4-year survival rate with Wilms Tumour in children is 80%.

Sources

Disclaimer: The content on this page is generic and shared only for informational and explanatory purposes. It is based on several secondary sources on the internet. As this content piece is not vetted by a medical professional, please consult a doctor before making any health-related decisions.