Wilms tumor (WT), named after Dr. Max Wilms, who first described it in 1899, is a rare kind of kidney cancer that primarily affects children. Also known as nephroblastoma, this malignancy typically arises in the kidneys and is most commonly diagnosed in children aged 3 to 4 years old. Although it’s rare, Wilms tumor is the most prevalent form of kidney cancer in children, comprising around 6-7% of all childhood cancers.
One of the hallmark symptoms of Wilms tumor is a firm, painless mass or swelling in the abdomen. Parents or caregivers may discover this mass while bathing or dressing the child. Other symptoms may comprise abdominal pain, blood in the urine, fever, nausea, and unexplained weight loss. However, it’s important to note that some children with Wilms tumor may not exhibit any noticeable symptoms until the tumor has grown significantly.
The exact cause of Wilms tumor remains unclear, but certain genetic & environmental factors may play a role in its development. In some cases, children with certain genetic syndromes, such as WAGR syndrome, Beckwith-Wiedemann syndrome, or Denys-Drash syndrome, may have a higher risk of getting a Wilms tumor. Additionally, there may be a familial predisposition in some cases, although the majority of Wilms tumors occur sporadically.
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Upon suspicion of Wilms’ tumor based on clinical presentation and imaging studies such as ultrasound, CT scan, or MRI, further diagnostic evaluation is typically performed to confirm the diagnosis. This may include a biopsy of the tumor tissue to examine it under a microscope and determine its characteristics. Once diagnosed, treatment for Wilms’ tumor typically involves a combination of surgery, chemotherapy, and sometimes radiation therapy.
Surgery is usually the primary treatment modality for Wilms’ tumor, with the goal of completely removing the cancer tumor while preserving as much healthy kidney tissue as possible. In some cases where the tumor is localized & has not spread, surgery alone may be curative. However, for tumors that are larger or have spread beyond the kidney, chemotherapy is often used before or after surgery to shrink the tumor and target any remaining cancer cells. Radiation therapy may also be utilized in certain cases to further reduce the risk of recurrence.
The prognosis for children with Wilms tumor has greatly improved over the years due to advances in treatment approaches. The overall survival rate for children with Wilms tumor is quite high, with approximately 9 out of 10 children surviving at least five years after diagnosis. However, long-term follow-up care is vital to monitor for any potential late effects of treatment and to address any ongoing medical or psychosocial needs of childhood cancer survivors.
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Wilms Tumor Definition
Wilms tumor, also known as nephroblastoma, is a kind of kidney cancer that mainly affects children. It is the most common abdominal malignancy in young children, accounting for nearly 90% of kidney tumors in this age group. Wilms tumor usually occurs before the age of 10, with a peak incidence between 2 and 3 years of age. It is slightly more common in girls than boys and in Black children than in Asian children.
Wilms tumor is named after Dr. Max Wilms, a German surgeon who first defined it in 1899. The tumor arises from immature renal cells that fail to differentiate into normal kidney tissue during embryonic development. These cells can form single or multiple tumors on one or both kidneys. The tumors can grow large and compress the surrounding organs, causing symptoms such as abdominal swelling, pain, blood in urine, fever and high blood pressure.
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Wilms Tumor Treatment
The main treatment options for Wilms tumor are surgery, chemotherapy and radiation therapy. The choice and sequence of these treatments depend on the stage and type of the tumor, as well as the risk group (low, intermediate or high) assigned to the patient based on certain criteria.
Surgery: Surgery is usually the first line of treatment for Wilms tumor. Surgery aims to remove the affected kidney (nephrectomy) and any nearby lymph nodes that may be involved. In some cases, partial nephrectomy (removing only part of the kidney) may be possible if the tumor is small and confined to one area. If feasible, surgery may also be done to remove any metastatic tumors (tumors that have spread to other organs).
Chemotherapy: It is the use of drugs to kill cancer tumors or stop them from growing. Chemotherapy may be prescribed before surgery (neoadjuvant) to shrink the tumor & make it more manageable to remove or after surgery (adjuvant) to kill any remaining cancer cells and prevent recurrence. Chemotherapy may also be given as a palliative treatment (to relieve symptoms & improve quality of life) for patients with advanced or inoperable tumors.
Radiation therapy: It is the use of high-energy beams or particles to destroy cancer cells or stop them from growing. Radiation therapy may be given after surgery (adjuvant) to kill any remaining cancer cells & prevent recurrence, especially for patients with high-risk tumors or unfavorable histology (certain types of cells that indicate a more aggressive tumor). Radiation therapy may also be prescribed as a palliative treatment for patients with advanced or inoperable tumors.
Supportive therapy: Supportive therapy is the use of medications and other interventions to manage the side effects & complications of cancer treatment, such as pain, nausea, vomiting, infection, bleeding, anemia, etc. Supportive therapy may also include psychological counseling, nutritional counseling, physical therapy, etc.
Cost of Wilms Tumor Treatment in India
The cost of Wilms tumor treatment in India varies depending on the type and stage of the tumor, the risk group of the patient, the choice and sequence of treatments, the type of hospital and room opted for, etc. However, the approximate cost ranges are as follows:
– Surgery: Rs. 2-3 lakhs
– Chemotherapy: Rs. 50-75 thousand per cycle (usually 6-8 cycles are required)
– Radiation therapy: Rs. 1-1.5 lakhs
– Supportive therapy: Rs. 10-20 thousand per month
Therefore, the total cost of Wilms tumor treatment in India may range from Rs. 2.22 lakhs to Rs. 2.96 lakhs for Indian patients and from USD 4500 to USD 5500 for international patients. These costs include preoperative diagnostic tests, surgery, postoperative care, chemotherapy, radiation therapy, medicines and hospital stay. However, these costs do not include any additional tests or treatments that may be required due to complications or recurrence.
Note: These costs are only indicative and may vary depending on various factors. It is advisable to consult your doctor and get a detailed estimate before starting any treatment.
Wilms Tumor Symptoms
Symptoms of Wilms tumor can vary depending on the size and location of the tumor, as well as whether it has spread to other parts of the body. Here’s a detailed explanation of the symptoms:
1. Abdominal Mass: The most common symptom of Wilms tumor is the presence of a firm, smooth mass or swelling in the abdomen. This mass may be noticed by parents or caregivers while bathing or dressing the child. It’s typically painless and may grow rapidly over time.
2. Abdominal Pain or Discomfort: Some children may face abdominal pain or discomfort. This pain can differ from mild to severe & may be constant or intermittent. It may be localized to the side or flank where the tumor is located.
3. Hematuria (Blood in Urine): Blood in the urine is another possible symptom of Wilms tumor. This blood may be observable to the naked eye, causing the urine to appear pink, red, or cola-colored, or it may only be detectable under a microscope.
4. High Blood Pressure (Hypertension): WT can sometimes cause high blood pressure, also known as hypertension. This occurs when the tumor presses on nearby blood vessels or affects the function of the kidneys, leading to increased blood pressure levels.
5. Fever: In some cases, children with Wilms tumor may develop a fever. This fever is often low-grade and may be accompanied by other symptoms like fatigue, loss of appetite, or weight loss.
6. Generalized Symptoms: As the tumor grows & potentially spreads to other parts of the body, children may experience more generalized symptoms such as fatigue, weakness, decreased appetite, and weight loss. These symptoms can be nonspecific and may mimic other childhood illnesses.
7. Other Signs of Metastasis: If Wilms tumor has spread (metastasized) to other parts of the body, additional symptoms may develop depending on the affected organs. For example, if the tumor spreads to the lungs, a child may experience difficulty breathing, coughing, or chest pain. If it spreads to the liver, jaundice (yellowing of the skin and eyes) may occur.
It’s important to note that only some children with Wilms tumor will experience all of these signs & symptoms, and some children may only experience symptoms at all once the tumor is quite large. Additionally, many of these symptoms are nonspecific and can be caused by other conditions, so parents and caregivers need to seek medical evaluation if they notice any concerning signs or symptoms in their child. Early detection and treatment of Wilms tumor can significantly improve outcomes.
Neuroblastoma Vs. Wilms Tumor
Neuroblastoma and Wilms tumor are both types of childhood cancers affecting different parts of the body. Here’s a breakdown of the key differences between the two:
1. Origin and Location:
– Neuroblastoma: This is a cancer that develops from immature nerve cells called neuroblasts. It typically starts in the adrenal glands (located on top of the kidneys) but can also occur in different areas of the abdomen, chest, neck, or pelvis.
– Wilms Tumor (Nephroblastoma): Wilms tumor originates from immature kidney cells. It generally affects children aged 3 to 4 and typically presents as a single tumor in one kidney, although it can develop in both kidneys simultaneously.
2. Age of Onset:
– Neuroblastoma: This cancer is most commonly diagnosed in children under the age of five, with the majority of cases occurring before the age of two.
– Wilms Tumor: The peak age for WT diagnosis is around 3 to 4 years old, although it can occur in older children as well.
3. Symptoms:
– Neuroblastoma: Symptoms may vary depending on the location and size of the tumor, but common signs are lump or swelling in the abdomen, bone pain, fever, weight loss, and changes in bowel or bladder habits.
– Wilms Tumor: Symptoms often include abdominal swelling or mass, abdominal pain, blood in the urine, fever, and high blood pressure.
4. Metastasis:
– Neuroblastoma: Neuroblastoma tends to spread (metastasize) to other parts of the body, such as the bones, bone marrow, liver, and lymph nodes, especially in advanced stages.
– Wilms Tumor: While Wilms tumor can spread to nearby lymph nodes, it usually remains localized to the kidneys in early stages. However, if left untreated, it can also spread to different organs like the lungs or liver.
5. Prognosis and Treatment:
– Neuroblastoma: The prognosis for neuroblastoma varies widely depending on factors such as age at diagnosis, stage of the cancer, and genetic characteristics. Treatment typically involves a mixture of surgery, chemotherapy, radiation therapy, immunotherapy, and targeted therapy.
– Wilms Tumor: The prognosis for Wilms tumor is generally favorable, especially when diagnosed early. Treatment involves surgery to remove the cancer tumor, followed by chemotherapy & sometimes radiation therapy. The survival rate for WT is high, particularly in cases where the cancer has not developed beyond the kidney.
In summary, while both neuroblastoma and Wilms tumor are childhood cancers, they arise from different types of cells in different locations within the body, have distinct age distributions, present with different symptoms, and may require different treatment approaches.
Wilms Tumor Prognosis
The overall survival rate for Wilms tumor in India ranges from 48% to 89%, depending on the study and the treatment protocol used. The key prognostic factors that influence survival include increased tumor volume, metastatic disease, unfavorable histology, non-compliance to treatment and non-relapse mortality. Non-relapse mortality refers to death from causes other than the tumor, such as infection, bleeding, organ failure or toxicity from treatment. Non-relapse mortality is high in India, ranging from 2.7% to 8.5%, due to various factors such as delayed diagnosis, poor nutrition, lack of supportive care and limited access to specialized centers.
To improve the outcome of Wilms tumor in India, there is a need for more awareness, early detection, standardized treatment protocols, multidisciplinary care and social support. There is also a need for more research and collaboration to understand the biology, epidemiology and genetics of this disease in the Indian population and to develop better therapies and strategies to overcome the challenges and barriers faced by patients and families.
Wilms Tumor Diagnosis
The diagnosis of Wilms tumor typically involves a combination of medical history assessment, physical examination, imaging tests, and biopsy. Here’s an overview of the diagnostic process:
1. Medical History and Physical Examination: The physician will begin by taking a complete medical history, asking about the child’s symptoms and any relevant family medical history. They will then do a physical evaluation, which may include feeling the abdomen for any lumps or swelling.
2. Imaging Tests: Various imaging tests are commonly used to diagnose Wilms tumor and determine its extent. These may include:
– Ultrasound: Ultrasound uses sound waves to develop pictures of the inside of the body. It is often the first imaging test performed to evaluate a suspected kidney tumor.
– CT (Computed Tomography) Scan: CT scans provide detailed cross-sectional images of the body and are used to evaluate the size & location of the tumor. They can also help decide if the cancer has spread to other body organs or lymph nodes.
– MRI (Magnetic Resonance Imaging): MRI scans use magnetic fields & radio waves to generate detailed pictures of the body. MRI may be used to further evaluate the extent of the tumor & its relationship to nearby structures.
– Chest X-ray: A chest X-ray may be conducted to check for any signs of metastasis (spread) to the lungs.
3. Biopsy: If imaging tests suggest the presence of a tumor, a biopsy is typically performed to confirm the diagnosis. During a biopsy, a tiny specimen of tissue is taken from the tumor & examined under a microscope by a pathologist to look for cancer tumors. In the case of Wilms tumor, the biopsy is usually obtained through a surgical procedure called a needle biopsy or a core biopsy.
4. Laboratory Tests: Blood & urine tests may be conducted to assess kidney function and look for markers associated with Wilms tumor, such as elevated levels of certain substances.
Once a diagnosis of WT is confirmed, further tests may be performed to determine the stage of the cancer and to guide treatment planning. These may include additional imaging studies, like bone scans or PET scans, to check for metastasis to other parts of the body.
It’s important to note that the diagnosis and management of Wilms tumor require a multidisciplinary approach involving pediatric oncologists, surgeons, radiologists, and pathologists to provide comprehensive care to the child.
Wilms Tumor Causes
Wilms tumor, also known as nephroblastoma, is a rare kind of kidney cancer that primarily affects children. The causes of Wilms tumor are not entirely understood, but research has identified various factors that may lead to its development:
1. Genetic Factors: There is medical evidence to suggest that genetic predisposition is vital in the development of Wilms tumor. Around 1-2% of cases are associated with specific genetic syndromes, such as:
– WAGR Syndrome: This syndrome is characterized by Wilms tumor, aniridia (absence of the iris), genitourinary abnormalities, and intellectual disability. It is caused by a deletion on chromosome 11p13 that includes the WT1 gene, which is also implicated in Wilms tumor development.
– Beckwith-Wiedemann Syndrome (BWS): BWS is characterized by overgrowth, macroglossia (enlarged tongue), omphalocele (abdominal wall defect), visceromegaly (enlargement of abdominal organs), and an increased risk of Wilms tumor. It is often associated with abnormalities in the regulation of imprinted genes on chromosome 11p15.5, including the IGF2 and H19 genes.
– Denys-Drash Syndrome: This syndrome is characterized by Wilms tumor, male pseudohermaphroditism (ambiguous genitalia), and nephropathy (kidney disease). Mutations in the WT1 gene cause it.
– Sporadic Mutations: In addition to these syndromic cases, sporadic mutations in genes such as WT1, CTNNB1, and WTX have been identified in non-syndromic Wilms tumors.
2. Abnormal Kidney Development: Wilms tumor typically arises from immature kidney cells that have failed to develop into mature, functioning kidney tissue. The exact reasons why these cells fail to develop properly are not fully understood, but disruptions in normal kidney development during fetal development may contribute to the formation of Wilms tumor.
3. Environmental Factors: While the role of environmental factors in Wilms tumor development is less clear compared to other cancers, some studies have suggested potential associations. Maternal exposure to certain substances during pregnancy, such as cigarette smoke, alcohol, or certain medications, has been investigated as potential risk factors for WT. However, more research is needed to establish a clear link between environmental exposures and WT development.
4. Epigenetic Changes: Epigenetic alterations, which affect gene expression without altering the DNA sequence, have been implicated in Wilms tumor development. Abnormal DNA methylation patterns and histone modifications in genes involved in kidney development and tumor suppression may contribute to the initiation and progression of Wilms tumor.
5. Fetal Anomalies: Certain congenital abnormalities and syndromes that affect fetal development, such as horseshoe kidney or renal agenesis, are associated with an increased risk of Wilms tumor. The underlying mechanisms linking these anomalies to WT development are not fully understood but may involve disruptions in normal kidney development pathways.
In summary, Wilms tumor is a complex disease with multifactorial etiology involving genetic, developmental, and environmental factors. Further research is needed to fully understand the interplay between these factors and their contributions to Wilms tumor development.
Conclusion
In conclusion, Wilms tumor, though rare, remains a significant concern in pediatric oncology. This type of kidney cancer predominantly affects children, often presenting with noticeable symptoms such as abdominal swelling or pain, blood in the urine, and fever. Early detection through routine medical examinations and imaging tests is crucial for prompt intervention and improved prognosis.
Advancements in medical technology and treatment protocols have significantly enhanced the survival rates and quality of life for children diagnosed with Wilms tumor. However, ongoing research is essential to refine existing therapies and develop novel approaches to address potential challenges, such as tumor recurrence and treatment-related side effects. With continued advancements in research and comprehensive care, the outlook for children facing this diagnosis continues to improve, offering hope for a brighter future beyond cancer.
Wilms tumor treatment often involves surgery, chemotherapy, and radiation therapy, along with additional expenses such as hospital stays and medications. These costs can quickly escalate, placing an immense financial strain on families. Crowdfunding provides a platform for individuals to reach out to a wide network of supporters, allowing them to raise the funds needed to cover treatment expenses.
