Wilms Tumor : Symptoms, Causes & Treatments - Medicover
Wilms tumour, also known as nephroblastoma, is a relatively rare but highly treatable type of kidney cancer primarily affecting children. It is the most common kidney cancer in children and typically occurs in early childhood, with most cases diagnosed before age 5, Wilms tumour arises from abnormal kidney cells and usually affects only one kidney, although it can occasionally occur in both kidneys simultaneously.
Types of Wilms tumour
Standard-Risk Wilms Tumor : This category includes favourable histology tumours and a low risk of recurrence. These tumours typically respond well to standard treatment approaches, which may involve surgery, chemotherapy, and sometimes radiation therapy.
High-Risk Wilms Tumor : High-risk Wilms tumours may fall into one or more categories:
Anaplastic Wilms Tumor : This aggressive subtype has a higher risk of recurrence and may require more intensive treatment.
Large Size : Tumors that are particularly large or have grown into nearby structures may be classified as high-risk.
Unfavourable Histology : Some tumours may have less responsive features to standard treatment.
Bilateral Wilms Tumor : When tumours are present in both kidneys, it is referred to as a bilateral Wilms tumour. A bilateral Wilms tumour requires careful evaluation and treatment planning due to the involvement of both kidneys.
Familial Wilms Tumor : In some cases, there may be a genetic predisposition to developing a Wilms tumour. Children with a family history of Wilms tumours or specific genetic syndromes, such as WAGR syndrome or Beckwith-Wiedemann syndrome, are at a higher risk.
Syndromic Wilms Tumor : Wilms tumour can be associated with a few hereditary diseases, such as WAGR syndrome (Wilms tumour, Aniridia, Genitourinary abnormalities, and intellectual Retardation) and Denys-Drash syndrome. These syndromes may increase the risk of developing Wilms tumour.
Spontaneous Wilms Tumor : Most Wilms tumours are sporadic and have no known underlying genetic cause or family history.
Symptoms of Wilms tumour include:
Abdominal Swelling or Mass : One of the most common symptoms, a painless lump or swelling in the abdomen, is often discovered by parents or caregivers while bathing or dressing the child. The stomach may appear asymmetrical or feel enlarged.
Abdominal Pain : Some children may experience abdominal discomfort or pain, which the pressure of the tumour on surrounding organs can cause.
Hematuria : Blood in the urine may lead to pink, red, or brownish-coloured urine. This symptom is less common but can indicate Wilms's tumour.
High Blood Pressure (Hypertension) : Wilms tumour can sometimes affect the blood vessels and lead to elevated blood pressure. This symptom may be detected during routine check-ups.
Fever : In some cases, children with Wilms tumours may develop an unexplained fever.
Loss of Appetite and Weight Loss:
Children may have a diminished appetite and lose weight unintentionally.
Fatigue : General tiredness or fatigue can be a nonspecific symptom associated with various medical conditions, including Wilms tumour.
Nausea and Vomiting:
Some children may experience vomiting and nauseous, which may be connected to the presence of the tumour.
Constipation : Tumors near the bowel may cause constipation or changes in bowel habits.
Respiratory Symptoms : If the tumour is large enough, it may cause compression of the lungs or other nearby structures, leading to respiratory symptoms such as coughing, wheezing, or difficulty breathing.
When to see a doctor?
Take a doctor's appointment if you are getting tired quickly and need to know why. If you observe the above Wilms tumour symptoms or during a blood test, your haemoglobin count has come low.
Causes of Wilms Tumor:
Genetic Mutations : Changes (mutations) in particular genes involved in kidney development and cell growth can increase the risk of Wilms tumour. These genetic mutations are not typically inherited from parents but occur spontaneously in the child's cells early in their development.
Genetic Syndromes : Some genetic syndromes are associated with an increased risk of developing Wilms tumour. For example, children with Beckwith-Wiedemann syndrome, WAGR syndrome (Wilms tumour, Aniridia, Genitourinary anomalies, and intellectual Retardation), and Denys-Drash syndrome are likelier to develop Wilms tumour.
Family History : While most cases of Wilms tumour occur sporadically (with no family history), a small percentage may have a genetic predisposition. A family history of Wilms tumour or other related conditions can increase the risk in some cases.
Kidney Development : Wilms tumour is believed to originate from immature kidney cells that did not develop properly during fetal development. Normal kidney cell differentiation and growth process changes may contribute to tumour formation.
Environmental Factors : While no specific environmental factors have been definitively linked to Wilms's tumour, exposure to certain substances or elements during pregnancy may potentially influence the risk. Research in this area is ongoing.
Wilms tumour Risk factors
Age : Wilms tumour most commonly occurs in children between 3 and 4, with a peak incidence around age 3. It is rare in older children and adults.
Genetic Syndromes : Certain genetic syndromes are linked to a higher risk of Wilms tumour.
These syndromes include:
Beckwith-Wiedemann syndrome : A genetic disorder characterized by overgrowth, large organs, and an increased risk of childhood cancers, including Wilms tumour.
WAGR syndrome (Wilms tumour, Aniridia, Genitourinary anomalies, and intellectual Retardation) : Individuals with WAGR syndrome are at an increased risk of Wilms tumour.
Denys-Drash syndrome : This rare genetic disorder is associated with kidney abnormalities and an elevated risk of Wilms tumour.
Family History : A family history of Wilms tumour or specific genetic syndromes can increase the risk. Children with a sibling or parent with Wilms tumour may have a slightly higher likelihood of developing the condition. Wilms tumour is significantly more frequent in girls than in boys.
Race and Ethnicity : Some studies suggest that Wilms tumour may be more common in African-American children than other racial and ethnic groups.
Birth Weight and Size : Large birth weight and some birth abnormalities, like malformations in the abdominal wall may be associated with a slightly increased risk of Wilms tumour.
Complications
Metastasis : Wilms tumour can spread (metastasize) to other body parts, such as the lungs, liver, and lymph nodes. Metastatic disease may require more aggressive treatment approaches.
Tumour Rupture : In some cases, the tumour may rupture or bleed, leading to abdominal pain, swelling, and possible infection. Rupture can also increase the risk of cancer cells spreading to other areas.
Hypertension (High Blood Pressure) : Wilms tumour can sometimes affect the blood vessels and lead to elevated blood pressure. Hypertension may require careful management and treatment.
Renal Dysfunction : Depending on the size and location of the tumour, it may affect the function of the affected kidney, leading to kidney dysfunction or failure.
Side Effects of Treatment : The treatments for Wilms tumours, such as surgery, chemotherapy, and radiation therapy, can cause various side effects. These may include fatigue, nausea, vomiting, hair loss, and increased infection susceptibility.
Diagnosis of Wilms tumour:
Physical examination and medical history taking:
Gather a detailed medical history, including any symptoms or changes in the child's health. Assemble a thorough medical history, assessing the child's overall health and identifying any signs of a kidney tumour.
Imaging Studies : Abdominal ultrasounds are frequently the first imaging tests.
It can help visualize the kidneys and any abnormal masses.
CT Scan (Computed Tomography) : A CT scan provides detailed cross-sectional images of the abdomen and can help determine the tumour's size, location, and extent.
MRI (Magnetic Resonance Imaging) : MRI may be used to assess further the tumour's characteristics and its relationship to surrounding structures.
Biopsy : In some cases, a biopsy may be conducted to establish the tumour's histological type and confirm the diagnosis.
A small sample of tissue is obtained. A pathologist removes tissue from the tumour and examines it under a microscope.
Laboratory Tests for Wilms tumour
Laboratory tests play a crucial role in the diagnosis and management of Wilms tumor, a rare kidney cancer primarily affecting children. These tests aid in confirming the presence of the tumor, assessing its stage, and guiding treatment decisions. Common laboratory tests for Wilms tumor include:
Urinalysis : This test examines urine for blood, abnormal proteins, and other markers that might indicate the presence of a kidney tumor.
Blood Tests : Blood tests measure kidney function, electrolyte levels, and specific biomarkers that can provide insights into the tumor's characteristics.
Imaging : While not a traditional laboratory test, imaging techniques like ultrasound, CT scans, and MRI are crucial for visualizing the tumor's location, size, and extent.
Biopsy : A tissue sample is taken from the tumor through a biopsy procedure. Laboratory analysis of this sample confirms the presence of Wilms tumor and helps determine its subtype.
Genetic Testing : Some cases of Wilms tumor have a genetic component. Genetic testing can identify specific genetic mutations that may influence treatment approaches.
Here is an overview of the treatment options for Wilms tumour:
Surgery (Nephrectomy) : The primary treatment for Wilms tumour is surgically removing the affected kidney (nephrectomy). In many cases, only the tumour and a portion of healthy kidney tissue are removed, allowing the child to retain kidney function. Minimally invasive surgical techniques may be used when appropriate.
Chemotherapy : Chemotherapy is often given before and after surgery. Preoperative chemotherapy (neoadjuvant chemotherapy) helps shrink the tumour, making it easier to remove during surgery. Postoperative chemotherapy (adjuvant chemotherapy) is administered to eliminate any remaining cancer cells and reduce the risk of recurrence. Chemotherapy drugs may include vincristine, dactinomycin, and doxorubicin.
Radiation Therapy : Radiation therapy may be recommended if there is a high risk of recurrence or if the tumour has spread to nearby lymph nodes or other areas. Radiation therapy uses high-energy beams to target and destroy cancer cells. It is typically used in specific cases and may be avoided in some low-risk situations.
Targeted Therapies : In some cases, targeted therapies or biological agents may target particular tumour cells' characteristics. These treatments are still being studied and may be combined with other therapies.
Clinical Trials : Participation in clinical trials may be considered, especially for high-risk cases. Clinical trials offer access to innovative treatments and contribute to advancing the understanding of Wilms tumour and its treatments.
Wilms tumour Dos and Don’ts
| Do's | Don'ts |
|---|---|
| Follow the prescribed treatment plan. | Don't skip or alter medications without medical advice |
| Attend all medical appointments | Don't delay or miss follow-up appointments |
| Maintain a healthy and balanced diet | Don't self-diagnose or rely on unverified treatments |
| Stay hydrated and get enough rest | Don't engage in strenuous activities without approval |
| Communicate openly with the healthcare team | Don't ignore new or worsening symptoms |
| Engage in age-appropriate physical activity | Don't neglect emotional well-being and support |
| Stay up-to-date on vaccinations | Don't avoid addressing concerns or questions |
| Seek support from family and friends | Don't isolate or withdraw from social interactions |
| Participate in supportive care services | Don't expose yourself to infectious environments |
Remember that these dos and don'ts should be discussed with the healthcare team
Wilms Tumor Care at Medicover Hospitals
At Medicover, we are proud to offer a dedicated team of experienced specialists and pediatric oncologists who provide comprehensive care for children diagnosed with Wilms tumours. Our medical experts are well-versed in managing Wilms tumour and its unique challenges. Here's what you can expect when seeking care for Wilms tumour at Medicover