Wilms Tumor, also known as nephroblastoma, is a rare kidney cancer that primarily affects children, typically those under the age of 5. It is the most common type of kidney cancer in pediatrics and accounts for about 90% of all childhood kidney tumors. The disease is named after Dr. Max Wilms, a German surgeon who first described the condition in the late 19th century.
Wilms Tumor usually occurs in just one kidney, but in about 5-10% of cases, it can affect both kidneys simultaneously (bilateral Wilms tumor). It arises from immature kidney cells that fail to develop properly during fetal growth, leading to the formation of a tumor.
Most children with Wilms Tumor present with an abdominal mass or swelling, often noticed by parents during bathing or dressing. Other signs may include abdominal pain, fever, blood in the urine (hematuria), high blood pressure, or loss of appetite.
The prognosis for Wilms Tumor has dramatically improved over the past decades due to advances in multimodal treatment, which often includes surgery, chemotherapy, and sometimes radiation therapy. With appropriate treatment, the survival rate exceeds 90% for localized tumors.
Early diagnosis and proper staging are essential for effective management. Genetic syndromes such as WAGR syndrome, Denys-Drash syndrome, and Beckwith-Wiedemann syndrome are known to increase the risk of Wilms Tumor, so children with these conditions require close monitoring.
Overall, Wilms Tumor is considered a highly treatable cancer in children, particularly when detected early and managed by a specialized pediatric oncology team.
The precise cause of Wilms tumor is not well established, but there are several factors that increase the risk of developing this condition. Research has shown that Wilms tumor often arises from genetic mutations during fetal development.
Genetic and Developmental Factors
Genetic Mutations: Some children may inherit mutations in specific genes that increase their risk of developing Wilms tumor. For example, mutations in genes related to kidney development, such as WT1, WT2, and FWT1, can lead to tumor formation. WT1 is the most commonly implicated gene, as it is essential for the normal development of the kidneys.
Congenital Syndromes: Certain congenital conditions and genetic syndromes increase the risk of Wilms tumor. These include:
WAGR Syndrome (Wilms tumor, Aniridia, Genitourinary anomalies, and Mental retardation).
Beckwith-Wiedemann Syndrome (a condition characterized by overgrowth and an increased risk of tumors).
Denys-Drash Syndrome (a genetic disorder that leads to kidney problems and Wilms tumor).
Family History: Although most cases of Wilms tumor occur sporadically, there is an increased risk in children with a family history of the condition. Some inherited mutations related to kidney development or other genetic disorders may make Wilms tumor more likely.
Risk Factors
Age: Wilms tumor is most commonly diagnosed in children under 5 years old. It is rare in adults and typically affects children between the ages of 3-4.
Sex: Wilms tumor is slightly more common in girls than in boys.
Ethnicity: Studies have shown that Wilms tumor is more common in African American children than in Caucasian children, although it is still relatively rare.
Family History of Cancer: If a child has a family member with Wilms tumor or another genetic condition associated with kidney cancer, they may be at higher risk of developing the disease.
Prematurity: Children born prematurely or with low birth weight may have a higher risk of developing Wilms tumor, as early developmental problems can lead to abnormal kidney growth.
The symptoms of Wilms tumor can vary depending on the size of the tumor, whether it has spread, and the individual child’s age. In some cases, Wilms tumor may not cause symptoms and can be discovered during a routine examination. However, the following symptoms are commonly associated with this condition:
The most common symptom of Wilms tumor is a palpable mass in the abdomen. This mass may be painless and may be noticed by the child’s parents or healthcare providers during routine physical examinations.
Children with Wilms tumor may experience abdominal pain or discomfort. The pain can be intermittent or persistent, and it may be caused by the tumor pressing against surrounding organs or structures.
In some cases, the tumor can cause blood in the urine (hematuria), which can be visible (gross hematuria) or detected through laboratory tests (microscopic hematuria). Blood in the urine may occur if the tumor is causing irritation or bleeding within the kidney.
A child with Wilms tumor may experience a low-grade fever, which can be associated with the tumor's growth or any associated infections.
Wilms tumor can sometimes cause high blood pressure, which is often a result of the tumor producing substances that affect blood vessels. High blood pressure may not be noticed by the child but can be detected during a physical exam.
Children with Wilms tumor may lose interest in eating and experience unexplained weight loss, which can be linked to the physical effects of the tumor or the overall impact on their health.
As the tumor grows, it can obstruct the kidneys or affect nearby organs, leading to nausea, vomiting, and a general feeling of illness.
Advanced stages of Wilms tumor can lead to swelling in the legs or face due to fluid buildup (edema), which can occur if the tumor affects the lymphatic system or veins.
Diagnosing Wilms tumor typically involves a combination of imaging studies, blood tests, and a biopsy to confirm the diagnosis and determine the extent of the disease. The diagnostic process usually includes:
A physical exam is the first step in diagnosing Wilms tumor. The healthcare provider may feel for an abdominal mass or other signs such as pain, swelling, or changes in the child’s vital signs.
Ultrasound: An abdominal ultrasound is often the first imaging test performed to detect an abdominal mass. It is non-invasive and provides real-time images of the kidneys and surrounding structures.
CT Scan (Computed Tomography): A CT scan is often used to determine the size and extent of the tumor and whether it has spread to other areas of the body, such as the lungs or lymph nodes.
MRI (Magnetic Resonance Imaging): An MRI provides detailed images of soft tissues and may be used to assess the tumor’s involvement with nearby structures and to plan surgery.
Complete Blood Count (CBC): A CBC can reveal anemia (a low red blood cell count) or signs of infection. It may also indicate if the kidney function is affected.
Urinalysis: A urinalysis can detect blood in the urine, a common sign of Wilms tumor.
Kidney Function Tests: Blood tests that assess kidney function may also be used, as Wilms tumor can affect the kidney's ability to filter waste products from the blood.
A biopsy may be performed to confirm the diagnosis of Wilms tumor. A sample of the tumor tissue is taken and examined under a microscope to determine the type of tumor and its stage. However, in many cases, the diagnosis can be made based on imaging tests alone, and a biopsy may not be required unless there is uncertainty about the diagnosis.
The treatment for Wilms tumor typically involves a combination of surgery, chemotherapy, and sometimes radiation therapy. The specific treatment plan depends on the tumor’s stage, the child’s age and overall health, and whether the tumor has spread to other parts of the body.
Nephrectomy: The primary treatment for Wilms tumor is surgery, usually to remove the affected kidney. This procedure is called a nephrectomy. In some cases, a partial nephrectomy may be performed if the tumor is confined to one part of the kidney.
Lymph Node Dissection: If the cancer has spread to the lymph nodes, they may also be removed during surgery to prevent further spread of the disease.
Chemotherapy is commonly used for Wilms tumor, either after surgery to prevent recurrence or as the primary treatment for tumors that are not surgically removable. Common chemotherapy drugs for Wilms tumor include vincristine, dactinomycin, and doxorubicin. Chemotherapy is usually administered in cycles, and the regimen depends on the tumor’s stage.
Radiation therapy may be used in certain cases, particularly if the tumor is large or has spread to other areas, such as the lungs or lymph nodes. External beam radiation is typically used to target the tumor and surrounding tissues to kill cancer cells and shrink the tumor.
In some cases, targeted therapy or participation in clinical trials may be an option. These therapies aim to specifically target the genetic mutations or signaling pathways that allow the tumor to grow, with the goal of limiting damage to healthy tissues.
While it is not possible to prevent Wilms tumor, early detection and treatment can significantly improve outcomes. Management focuses on treating the disease, monitoring for recurrence, and addressing any long-term effects of treatment.
Routine pediatric check-ups are essential for detecting abnormalities early. Parents should be vigilant for signs of abdominal swelling, pain, or changes in a child’s health and seek medical attention promptly if these symptoms appear.
Children treated for Wilms tumor require long-term follow-up care to monitor for recurrence, growth, and development. This often includes regular imaging studies, blood tests, and physical exams to ensure the child is healthy and to detect any late effects of treatment.
Managing the psychological and emotional well-being of the child and their family is an important aspect of treatment. Support groups, counseling, and pediatric rehabilitation services can help children cope with the disease and the impact of treatment on their lives.
Although many children recover fully from Wilms tumor, there are potential complications, especially if the tumor is diagnosed at an advanced stage or if the treatment causes side effects:
Recurrence: Wilms tumor can recur, particularly if it was not completely removed during surgery or if the cancer was aggressive.
Growth and Developmental Delays: Some children may experience delays in growth or cognitive development, especially if they receive extensive radiation therapy.
Long-term Side Effects of Treatment: Chemotherapy and radiation therapy can have long-term effects on fertility, organ function, and overall health, especially in survivors treated at a young age.
Emotional and Psychological Impact: Coping with cancer treatment can be emotionally challenging for both children and their families. Survivors may experience anxiety, depression, or PTSD after treatment.
Living with Wilms tumor involves ongoing medical care, monitoring, and support:
Children who survive Wilms tumor require long-term follow-up care to monitor for recurrence, manage any treatment side effects, and assess overall health. Regular visits with a pediatric oncologist, imaging tests, and blood work are important for detecting problems early.
Survivors may benefit from educational support and counseling to help them adjust to the physical and emotional changes that come with recovery. Peer support groups for children and families affected by cancer can also provide comfort and guidance during and after treatment.
Maintaining a healthy lifestyle with a balanced diet and regular exercise is important for long-term recovery. Physical therapy and other rehabilitation services may be needed if there are any physical limitations due to treatment.
Wilms tumor is a rare type of kidney cancer that primarily affects children, usually under the age of 5. It begins in the cells of the kidney and is the most common type of kidney cancer in children. Wilms tumor typically develops in one kidney, but in some cases, it can affect both kidneys. It is named after the German surgeon Max Wilms, who first described the tumor in the 1890s.
The exact cause of Wilms tumor is not fully understood, but it is believed to be related to abnormal development of kidney cells during fetal growth. While most cases of Wilms tumor occur sporadically, there are certain genetic conditions that can increase the risk, including:
Wilms tumor gene (WT1) mutations
WAGR syndrome (Wilms tumor, Aniridia, Genitourinary anomalies, and Retardation)
Beckwith-Wiedemann syndrome
Denys-Drash syndrome
While these genetic conditions increase the likelihood of Wilms tumor, most
cases occur without a family history or underlying syndrome.
Symptoms of Wilms tumor may vary, but common signs include:
A noticeable lump or mass in the abdomen
Abdominal pain or swelling
Fever
Hematuria (blood in the urine)
High blood pressure (hypertension)
Loss of appetite
Weight loss
In some cases, there may be no symptoms until the tumor grows large enough to be
detected.
Diagnosis of Wilms tumor usually involves several steps:
Physical examination: The doctor may feel for an abdominal mass.
Imaging tests: A combination of ultrasound, CT scans, or MRI may be used to visualize the tumor and check its size and spread.
Biopsy: In some cases, a biopsy is taken to confirm the diagnosis and determine the type of tumor.
Blood and urine tests: These can help assess kidney function and detect signs of abnormal kidney cells.
Wilms tumor is staged based on the extent of the disease and how far it has spread. The stages range from Stage I to Stage V:
Stage I: The tumor is confined to the kidney and can be completely removed through surgery.
Stage II: The tumor has spread to surrounding tissues but can still be removed.
Stage III: The tumor has spread to nearby lymph nodes or organs within the abdomen.
Stage IV: The tumor has spread to distant organs such as the lungs, liver, or bones.
Stage V: Both kidneys are affected by tumors.
Staging helps determine the best course of treatment and gives information about the prognosis.
Treatment for Wilms tumor typically involves a combination of surgery, chemotherapy, and sometimes radiation therapy, depending on the stage of the disease:
Surgery: The primary treatment for Wilms tumor is the removal of the affected kidney (nephrectomy). If the tumor is large, a portion of the kidney may be removed instead of the entire organ.
Chemotherapy: Drugs are used to kill cancer cells and are often administered before or after surgery to reduce the size of the tumor or address any remaining cancer cells.
Radiation therapy: May be used for more advanced stages or when
the tumor cannot be fully removed surgically.
The treatment approach will vary based on the stage and individual patient
factors.
Currently, there is no known way to prevent Wilms tumor, as the exact cause is not fully understood. However, early detection through regular checkups and awareness of symptoms can lead to prompt diagnosis and treatment, improving the chances of a successful outcome. Children with known genetic conditions that increase the risk of Wilms tumor may require more frequent monitoring.
The prognosis for children with Wilms tumor has greatly improved over the years, with survival rates for localized tumors exceeding 90%. The prognosis depends on various factors, including:
The stage of the tumor at diagnosis
The child's age and overall health
The tumor's response to treatment
Whether the cancer has spread to other parts of the body
Early diagnosis and successful treatment typically lead to long-term survival,
though the prognosis is less favorable for children with metastatic disease or
those with genetic syndromes associated with Wilms tumor.
Potential complications of Wilms tumor and its treatment may include:
Kidney function problems: Removal of a kidney or radiation therapy can affect kidney function, especially if both kidneys are affected.
Infection: Chemotherapy weakens the immune system, making children more susceptible to infections.
Growth problems: Some children may experience developmental delays or growth issues, especially if the tumor was treated with radiation.
Secondary cancers: In rare cases, children who received radiation therapy for Wilms tumor may develop secondary cancers later in life.
Follow-up care is essential for children who have been treated for Wilms tumor. This includes:
Regular imaging: To check for signs of recurrence, especially in the first few years after treatment.
Kidney function tests: To monitor the health of the remaining kidney.
Monitoring growth and development: Since some treatments can affect a child’s growth or cognitive development.
Psychosocial support: Counseling or support groups for the child
and family to address emotional challenges after treatment.
Follow-up care ensures that any issues are identified early, and ongoing
monitoring helps ensure the best possible outcome.
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