Wilms Tumour

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Overview

Wilms tumour or nephroblastoma is a rare type of kidney cancer that is more prevalent among children. Wilms tumours account for approximately 9 out of 10 kidney malignancies seen in children.

In most cases, nephroblastoma can be treated with positive clinical outcomes.

Symptoms

One of the first symptoms associated with Wilms tumour is abdominal swelling. The symptoms of Wilms tumour are easily confused with that of other less severe health conditions. Therefore, parents must be mindful of the symptoms that are associated with this condition and report them to the doctor immediately.

Presence of abdominal mass
Pain and discomfort in the abdominal region
Loss of appetite
Nausea and vomiting
Extreme weakness and fatigue
Fever
Blood in urine
Constipation
Increased blood pressure
Shortness of breath
One side of the body grows faster than the other

Courses

The exact cause of Wilms tumour is unknown. However, a few risk factors for Wilms tumour have been identified:

Certain Genetic Disorders: Those born with certain genetic disorders have a higher risk of developing Wilms tumour. The key genetic disorders whose incidence is linked with Wilms tumour include:
- Denys-Drash syndrome
- Trisomy 18 or Edward’s syndrome
- Beckwith-Wiedemann syndrome
- Sotos syndrome
- Simpson-Golabi-Behmel syndrome
- Frasier syndrome
- Li-Fraumeni syndrome
- Bloom syndrome
- Perlman syndrome
- WAGR syndrome
Age: This cancer type is more prevalent in children aged four and below. It is extremely rare in adults.
Family History of Wilms Tumour: Having a family history of Wilms tumour increases one’s risk of developing this disease.

Diagnosis

There are different tests that a doctor may recommend while diagnosing Wilms tumour:

a. Physical Exam and Medical History Assessment: Before suggesting any test, the doctor thoroughly examines the patient for signs of Wilms tumour, which could be abdominal swelling, pain, high blood pressure, abnormal growth, etc. He/she may also take a note of the patient’s complete medical history.

b. Imaging Tests: If the doctor suspects Wilms tumour, he/she may recommend imaging tests for more information on the tumour growth. Abdominal ultrasound scan is one of the first imaging tests suggested along with MRI scans, PET CT scans, bone scans, etc. These tests created detailed images of the internal structures that will help doctors in arriving at an accurate diagnosis. These tests are also important for checking if cancer has spread to nearby organs.

Imaging tests are frequently written throughout the treatment for disease staging, treatment planning, therapy monitoring and disease-restaging.

c. Biopsy: A biopsy is recommended for the definitive diagnosis of Wilms tumour. During this procedure, a tissue sample from the suspected area is collected and checked for the presence of cancer cells.

d. Blood and Urine Tests: There are no blood tests recommended for the diagnosis of Wilms tumour. They may be only written to check the overall health of the patient, the liver and kidney functions, etc.

Treatment

The treatment planning for Wilms tumour is devised upon considering a multitude of factors namely, the stage of the disease, the size of the tumour and the overall condition of the patient. The main treatment options available for Wilms tumour include surgery, radiation therapy and chemotherapy.

a. Surgery: Surgery is the primary treatment for Wilms tumour. This procedure may be combined with other treatment options, namely radiation therapy and chemotherapy to support better treatment response. Depending on the stage of the disease, one of the following surgeries may be recommended for the successful management of Wilms tumour:

1. Partial Nephrectomy: During a partial nephrectomy, the surgeon removes the tumour along with a small amount of surrounding healthy tissue.
2. Radical Nephrectomy: Radical nephrectomy refers to the removal of the entire kidney along with surrounding tissues and lymph nodes.

b. Radiation Therapy: Radiation therapy is a localised therapy that destroys cancer cells using high-energy radiation beams. Radiation therapy may be administered before the surgery to shrink the tumour and after the surgery to kill the remaining cancer cells.

c. Chemotherapy: Chemotherapy is a systemic therapy recommended for Wilms tumour. It uses powerful anticancer drugs to destroy the cancer cells throughout the body. In a few cases, more than one chemo drug may be used to destroy the cancer cells.

Usually, chemotherapy is administered before the surgery to shrink the tumour (neoadjuvant chemotherapy) and after the surgery to destroy the remaining cancer cells and reduce the risk of recurrences.

Frequently Asked Questions

1. Can Wilms tumour be treated?

Wilms tumour is one of the highly treatable cancers. Most of the children with Wilms tumours go on to have normal lives after the treatment.

Nevertheless, it is important for parents to not ignore any symptoms that could be associated with this disease. Ignoring symptoms may lead to delayed diagnosis, which could further lead to poor clinical outcomes.

2. My kid has a mass in the abdomen region. What should I do?

Firstly, do not panic. Secondly, do not make any delay. Immediately consult your physician and get necessary tests done to find out the cause. Make sure to follow all the instructions given by the doctor.

If it is Wilms tumour or any other condition, ensure to ask the doctor as many questions as possible. This will give you a better understanding of the disease and help you know what to expect. Along with this, you can also do your bit of research to understand various treatment options available and the crucial aspects of follow-up care.

3. Can Wilms tumour come back?

Yes, in some cases, Wilms tumour can come back. However, if they are caught on time, they can be treated successfully.

Strict follow-up care and monitoring after the treatment are extremely important in reducing the risk of a relapse. Therefore, the parents or caregivers need to ensure that the patients keep up their follow-up appointments and all instructions given by the doctors are ardently followed.

4. Can Wilms tumour be prevented?

The risk factors (age, genetic disorders, family history, etc.) that are associated with Wilms tumour cannot be controlled. Also, this condition is not associated with any lifeclass-related risk factors. Therefore, there are no known ways to prevent or control the risk of Wilms tumour presently.

However, those identified as high-risk individuals for this condition can seek doctors’ help to understand the best practices that can help them in reducing the risk of this disease to some extent.