Wilms Tumour Treatment in Delhi, India

Wilms tumour, also known as nephroblastoma, is a rare kidney cancer primarily affecting children. It is the most common kidney cancer in children, usually diagnosed in those aged 3 to 4, and becomes much less common after age 5. While it typically occurs in one kidney, it can sometimes affect both. Advancements in diagnosis and treatment have significantly improved the prognosis for children with Wilms tumour. With proper treatment, the outlook for most children with this condition is positive.

The exact cause of Wilms tumour remains unknown. However, researchers have identified some factors that may increase the risk of developing it:

• Genetics: Inherited gene mutations can play a role in some Wilms tumour cases. These mutations can be passed down from a parent or occur spontaneously during development. Specific genetic conditions like Beckwith-Wiedemann syndrome, Denys-Drash syndrome, and WAGR syndrome increase the risk of Wilms tumour.
• Sporadic Mutations: In most Wilms tumour cases, the gene mutations involved are not inherited and occur randomly during foetal development.

Wilms tumour may not cause any symptoms in its early stages. However, as the tumour grows, some potential signs to watch for include:

• A painless lump in the abdomen (belly): This is often the first noticeable symptom. The lump may feel firm or rubbery and might increase in size over time.
• Abdominal pain or swelling: The growing tumour can cause discomfort or bloating in the abdomen.
• Blood in the urine (hematuria): Blood in the urine (hematuria) can occur if the tumour affects the urinary tract.
• High blood pressure: Wilms tumour can sometimes lead to an increase in blood pressure.
• Fever: Unexplained fever can be a sign of Wilms tumour or other childhood conditions.
• Feeling generally unwell (malaise): Children with Wilms tumour might experience fatigue, loss of appetite, or nausea.

These symptoms can also be caused by other conditions. If you notice any of these signs in your child, it's crucial to consult a Wilms Tumour doctor for proper diagnosis. Early detection is essential for successful Wilms tumour treatment.

Diagnosing Wilms tumour typically involves a multi-step process to confirm the presence and extent of the cancer. Here's a breakdown of the usual course:

Medical History and Physical Exam

The doctor will discuss the child's symptoms and family history (including any genetic conditions) and perform a physical examination focusing on the abdomen to feel for any lumps or swelling.

Imaging Tests

Imaging tests are crucial for visualising the kidneys and any potential tumours. Common tests used include:

• Ultrasound: This painless test uses sound waves to create an image of the abdomen and kidneys. It's often the first imaging test performed due to its non-invasive nature.
• CT Scan: A CT scan uses X-rays to create detailed cross-sectional images of the abdomen and surrounding structures. It can provide a clearer picture of the tumour size, location, and any potential spread to other organs.
• MRI Scan: An MRI scan uses strong magnetic fields and radio waves to generate detailed images of the body, including the kidneys and surrounding tissues. It might be helpful in differentiating Wilms tumour from other types of abdominal masses.

Blood and Urine Tests

These tests assess overall health and kidney function. Blood tests might check for complete blood count, electrolytes, and liver function. Urine tests can look for blood or other abnormalities.

Biopsy (optional)

In some cases, a biopsy might be recommended to confirm the diagnosis definitively. A small tissue sample is extracted from the tumour, usually with ultrasound or CT guidance, for examination under a microscope by a pathologist. This helps determine the type of Wilms tumour and guide treatment decisions.

If Wilms tumour is diagnosed, further tests are typically done to determine the stage (extent) of the cancer. This helps with treatment planning and prognosis. Staging may involve additional imaging tests like chest X-ray or CT scan to see if the cancer has spread to the lungs.

Wilms tumour is staged based on the extent of the disease at the time of diagnosis and after surgery. The stages range from I to V, with each stage indicating a progressively more advanced disease. This staging system helps doctors plan the most appropriate treatment approach and estimate the prognosis (chance of cure). Here's a breakdown of the Wilms tumour stages:

Stage I

• The tumour is confined entirely within one kidney and has been completely removed by surgery.
• There is no evidence of cancer spread to lymph nodes or other organs.
• This is the earliest and most favourable stage with a very high cure rate.

Stage II

• The tumour has grown beyond the original kidney but remains within the abdomen.
• It might involve:
• Extending into surrounding fatty tissue.
• Infiltrating the kidney capsule (the outer layer of the kidney).
• Reaching the renal vein (the main vein draining the kidney).
• However, the entire tumour is still successfully removed by surgery, with no apparent cancerous tissue left behind.
• There is no lymph node involvement or spread to other organs.

Stage III

• Complete surgical removal of the tumour is not possible due to several factors:
• The tumour is too large or complex to remove entirely.
• Cancerous tissue remains in the abdomen after surgery (residual tumour).
• Lymph nodes in the abdomen contain cancer cells.
• There's still no spread to distant organs at this stage.

Stage IV

• The cancer has spread beyond the abdomen and kidneys to distant organs through the bloodstream. Common sites for metastasis (spread) include:
• Lungs
• Liver
• Bones
• Involvement of lymph nodes beyond the abdomen also falls under Stage IV.

Stage V

• This stage signifies bilateral Wilms tumour, where both kidneys are involved with tumours at the time of diagnosis.
• Each kidney is individually staged (can be Stage I-IV) based on the extent of tumour involvement in that specific kidney.

Understanding the stage of Wilms tumour is crucial for determining the appropriate treatment plan and prognosis. Early-stage tumours (stages I and II) generally have a better prognosis and may require less intensive treatment compared to advanced-stage tumours (stages III, IV, and V).

Wilms tumour treatment typically involves a combination of surgery, chemotherapy, and sometimes radiation therapy. The specific approach depends on various factors, including the stage of the cancer, the child's age and overall health, and the type of Wilms tumour. Here's a breakdown of the main treatment options:

Surgery

• Primary Aim: The first line of treatment for most Wilms tumours aims to remove the entire tumour whenever possible.
• Types of Surgery:
• Nephrectomy: Removing the affected kidney entirely. In some cases, if the tumour is small and confined to one kidney, a partial nephrectomy (removing only the part of the kidney containing the tumour) might be an option to preserve healthy kidney tissue.
• Biopsy: In some situations, a biopsy might be performed before definitive surgery to confirm the diagnosis and guide treatment decisions.

Chemotherapy

• Purpose: Chemotherapy uses powerful medications to destroy cancer cells throughout the body.
• Before Surgery (Neoadjuvant Chemotherapy): To shrink the tumour and make surgery easier and more effective.
• After Surgery (Adjuvant Chemotherapy): To reduce the risk of cancer recurrence by eliminating any microscopic cancer cells remaining after surgery.
• Administration: Chemotherapy medications are typically administered through an intravenous (IV) line inserted into a vein.

Radiation Therapy

• Purpose: Radiation therapy uses high-energy X-rays or other forms of radiation to kill cancer cells.
• When It's Used:
• After Surgery: For high-risk tumours to destroy any remaining cancer cells.
• Inoperable Cases: When surgery is not possible or too risky.
• Metastatic Tumours: To treat tumours that have spread to other organs.

Supportive Care

Various treatments to manage side effects from cancer treatments and improve the child's overall well-being during the treatment course.

The treatment plan is tailored to each child's specific situation and often involves a multidisciplinary team of specialists, including paediatric oncologists, surgeons, radiation oncologists, nurses, and supportive care providers. Regular follow-up is crucial to monitor for any signs of recurrence and manage any long-term side effects of treatment.

While Wilms tumour is a serious condition, significant advancements in treatment have led to very high cure rates,especially for early-stage tumours. However, there are still some potential complications associated with Wilms tumour and its treatment:

• Relapse: This refers to the cancer returning after treatment. The risk of relapse depends on the stage of the cancer and other factors. Regular follow-up care is crucial for early detection of any recurrence.
• Side effects from treatment:
• Surgery: Potential side effects of surgery can include pain, infection, bleeding, and damage to nearby organs or nerves. The extent of side effects depends on the type and complexity of surgery performed.
• Chemotherapy: Common side effects of chemotherapy include nausea, vomiting, fatigue, hair loss,increased risk of infection, and mouth sores. These side effects are usually temporary and manageable with medication and supportive care.
• Radiation therapy: Radiation therapy can cause side effects like fatigue, skin irritation, and digestive problems in the area exposed to radiation. These effects typically go away gradually after treatment completion.
• Kidney problems: Depending on the extent of surgery or damage caused by the tumour, there might be a risk of long-term kidney problems, such as high blood pressure or decreased kidney function.
• Increased risk of other cancers: Children who have been treated for Wilms tumour have a slightly increased risk of developing other cancers later in life. Regular follow-up care with a doctor is important for monitoring this risk.

There are no specific measures to prevent Wilms tumour, as the exact cause is not well understood. However, early detection and monitoring can help manage the condition more effectively:

• Genetic Counselling: Families with a history of Wilms tumour or associated genetic conditions may benefit from genetic counselling.
• Regular Check-Ups: Children with genetic syndromes or congenital anomalies associated with a higher risk of Wilms tumour should have regular medical check-ups.
• Awareness of Symptoms: Early recognition of symptoms, such as abdominal swelling or pain, can lead to earlier diagnosis and treatment.

While prevention may not be possible, these steps can aid in early detection and improve treatment outcomes for affected children.

Can environmental factors contribute to the development of Wilms tumour?

There is no clear evidence that environmental factors contribute significantly to the development of Wilms tumour. Most cases are believed to be due to genetic mutations that occur randomly.

Can Wilms tumour recur after treatment?

Yes, Wilms tumour can recur after treatment. Regular follow-up care is essential to monitor for any signs of recurrence and to manage any long-term effects of treatment.

Is it possible for children to lead normal lives after treatment for Wilms tumour?

Many children can lead normal, healthy lives after treatment for Wilms tumour, especially if the cancer is detected early and treated effectively. Regular medical check-ups are important to ensure ongoing health.

Are there any dietary restrictions for children undergoing treatment for Wilms tumour?

There are generally no specific dietary restrictions, but a balanced, nutritious diet is crucial to support the child's overall health and recovery. A dietitian can provide personalised advice based on the child's needs.

Can adults develop Wilms tumour?

Wilms tumour is very rare in adults. It primarily affects young children, typically those aged 3 to 4 years old.

How does Wilms tumour develop?

Wilms tumour develops from immature kidney cells that begin to grow uncontrollably, forming a mass. The exact cause of these changes is not fully understood, but genetic factors play a significant role.

What follow-up care is necessary after Wilms tumour treatment?

Follow-up care usually includes regular physical exams, imaging tests, and laboratory tests to monitor for recurrence and manage any late effects of treatment.

Are there any long-term side effects of Wilms tumour treatment?

Long-term side effects can include issues with kidney function, growth, heart health, and secondary cancers. The risk depends on the treatment used and the individual child's health.

How is the quality of life affected after Wilms tumour treatment?

Many children return to normal activities after treatment, but some may experience physical or emotional challenges. Supportive care and rehabilitation can help address these issues.

What support services are available for families dealing with Wilms tumour?

Support services include counselling, support groups, social work services, and resources for financial assistance. These services help families cope with the emotional and practical aspects of the diagnosis.

How can parents prepare their child for Wilms tumour treatment?

Parents can prepare their child by explaining the treatment process in an age-appropriate way, maintaining routines, and providing emotional support. Involving child life specialists can also be helpful.

What lifestyle changes might be needed during and after treatment for Wilms tumour?

During treatment, children may need to avoid certain activities to reduce the risk of infection or injury. After treatment, regular follow-up care and maintaining a healthy lifestyle are important for long-term health.

Are there any specific exercise recommendations for children undergoing Wilms tumour treatment?

Exercise recommendations depend on the child's overall health and treatment plan. Gentle, age-appropriate activities can help maintain physical fitness and emotional well-being.

How can siblings and other family members be supported during the treatment process?

Providing clear information, involving them in the care process, and ensuring they have opportunities to express their feelings can help support siblings and other family members. Counselling and support groups can also be beneficial.

What resources are available for financial assistance during Wilms tumour treatment?

Financial assistance resources may include hospital social work departments, non-profit organisations, and government programs designed to help families with the costs associated with cancer treatment.

How can parents monitor their child’s health post-treatment for Wilms tumour?

Parents should keep regular follow-up appointments, watch for any new or recurring symptoms, and maintain open communication with their child’s healthcare team to monitor health post-treatment.

What is the role of genetic counselling in Wilms tumour cases?

Genetic counselling can help families understand the potential genetic risks, guide decisions about genetic testing, and provide information on the likelihood of recurrence in future pregnancies or siblings.

Reviewed By Dr. Sanjay Gogoi, Principal Director - Urology, Kidney Transplant & Uro Oncology on 27 Aug 2024.