Childhood Kidney Cancer – Spilling The Beans

Kidney cancer accounts for 7% of all childhood cancer, hence making it a rare condition. Kidney cancer occurs when kidney cells begin to grow and divide uncontrollably that it can impair function and lead to tumour formation. It can be a terrifying experience for parents to see their child struggling with this life-threatening illness.

Childhood kidney cancer can be effectively managed with the right approach, allowing children to reclaim their health and live their lives. Awareness on the subject will provide the best possible outcome for a child. While hospitals provide expert medical care and treatment, parents can offer their child comfort and support.

In conjunction with Rare Disease Day, Sunway Medical Centre, Sunway City sheds light on childhood kidney cancer to better equip parents.

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Wilms’ tumour
Wilms’ tumour is the most common type of paediatric kidney cancer. They are caused by mutations in kidney cells that typically occur after birth and usually affects one kidney. However, in 5% to 7% of cases, it can be found in both kidneys and the majority of cases are sporadic.

The cause of most cases of childhood cancers unknown. However, there are certain inherent genetic disorders that are present in a child that increases their risk of developing Wilms’ tumour. It can either occur in isolation such as aniridia (absence of iris in the eye), hemihypertrophy or overgrowth of one side of the body, or as part of a syndrome (a collection of signs and symptoms that occur together). One of these syndromes is WAGR, which is the acronym for Wilms tumour, aniridia, genitourinary malformation, and range of developmental delays. Patients with WAGR syndrome have a 45% to 60% chance of developing Wilms’ tumour.

Dr Eni Juraida Abdul Rahman, Consultant Paediatrician and Paediatric Haematologist & Oncologist at Sunway Medical Centre, Sunway City explains, Wilms’ tumour is a cancer that occurs in young children usually under the age of 5 years. “As the child grows older, the risk of them developing Wilms tumour is lesser, and we hardly ever see Wilms’ tumour in adults.”

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Symptoms

1. Painless swelling in the abdomen. Occasionally noted by parents while bathing the child. The tumour may cause the abdomen to swell, causing discomfort
2. Haematuria or presence of blood in urine
3. Hypertension. Wilms’ tumour can cause high blood pressure
4. Fever
5. Loss of appetite. Children may experience a loss of appetite, resulting in weight loss
6. Pain in the abdomen especially if there is bleeding within the tumour causing a sudden increase in size of the mass
7. Generally feeling unwell
8. Cough and shortness of breath if the tumour has spread to the lungs

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Treatment
According to Dr Eni, in most cases, by the time the child arrives for a diagnosis, the tumour has grown to a large size, and the kidney is effectively non-functional.

If kidney tumour is suspected, the patient will undergo blood tests to look into the general health of the patient, haemoglobin level and kidney function. Radiological examination are also performed such as an ultrasound of the abdomen, CT scan to further define the tumour, and for staging.

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Tumour staging

1. Tumour is within the kidney
2. Tumour is still within the kidney but has involved surrounding fatty tissue and blood vessels but can still be completely removed
3. Very large tumours that has crossed the midline of the body
4. Tumour has spread to distant sites usually the lungs, liver or bone
5. Tumour involving both kidneys

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Subsequently, depending on the stage of the tumour, the patient will undergo:

• Surgery or nephrectomy to remove the affected kidney. This is usually done upfront for stage I or II tumours or delayed for stage III disease. Surgery is the mainstay of treatment and all children with undergo surgery. What will cure the child is surgery, and not the chemotherapy, Dr Eni explains. All efforts will be futile if the tumour grows back without a follow-up surgery after chemotherapy. Partial nephrectomy or renal-sparing surgery is reserved for bilateral tumours
• Chemotherapy where drugs are given that will kill the cancer cells. This can be:
  • Adjuvant: Given after surgery to kill any remaining cancer cells. For stage I and II tumours
  • Neoadjuvant. Given before surgery to reduce the size of the tumour and kill cells that have spread to other areas. Chemotherapy is given upfront for stage III tumours because the tumour is very big. The aim is to shrink the tumour to allow surgery to be carried out safely
• Radiotherapy in some patients depending on what the tumour looks like microscopically, how far it has spread outside the kidney or if it has spread to distant organs such as the lung and has not completely resolved with chemotherapy

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Outcome
The outcome for Wilms’ tumour is good. “For stage 1 Wilms’ tumour, the cure rate is 95%. Similarly, for stages II to IV, if there are no unfavourable features in the histology, a 4 year survival rate of 90% can be expected,” Dr Eni elaborates.

Prevention
“Since we don’t know the cause, it is difficult to have any preventive measure. However, for children who have abnormalities such as WAGR, surveillance ultrasound of the abdomen are carried out periodically e.g. every 6 months to detect the occurrence of this tumour. Parents are also counselled to be more vigilant regarding symptoms and signs. It is the hospital’s responsibility to increase public awareness on what are the common symptoms and signs of childhood cancer,” Dr Eni shares.

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Though there aren’t any preventive measures, these are things to look out for:

1. Most tumours are discovered incidentally. It is a good idea to keep an eye out for lumps when parents are dressing or bathing their child. It will be obvious when one side of the stomach looks bigger than the other.
2. Presence of blood in the urine. This symptom is commonly known as haematuria. This shows that the bleeding could be coming from the kidney, prostate, or bladder.
3. Loss of appetite and weight. Tumours that arise from the left kidney, will compress the stomach as it grows bigger leading to a sense of fullness when the child eats.
4. Big birth weights with hemihypertrophy. They have a higher chance of developing a renal tumour. Hence, should be screened periodically.
5. Advising mothers to exercise caution when taking any health supplements because what we consume is usually metabolised by the liver and some of these substances have been known to affect the function of the liver and the kidney.

Sunway Medical Centre, Sunway City believes that parents should be aware of common childhood cancer symptoms and signs, and knowing when to seek medical attention. With the advancement of medical research and treatment, Sunway Medical Centre ensures high prognosis for children with kidney cancer.

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