Wilms Tumor : Symptoms, Causes & Treatments - Medicover

Types of Wilms tumour

• Standard-Risk Wilms Tumor : Includes favourable histology and low recurrence risk, often treated with surgery, chemotherapy, and sometimes radiation.
• High-Risk Wilms Tumor : Can include:
  • Anaplastic subtype : More aggressive, requiring intensive treatment.
  • Large size or involvement of nearby structures.
  • Unfavourable histology : Less responsive to standard treatment.
• Bilateral Wilms Tumor : Involves both kidneys, requiring careful treatment planning.
• Familial and Syndromic Wilms Tumor : Genetic predisposition linked to conditions like WAGR syndrome and Denys-Drash syndrome.
• Spontaneous Wilms Tumor : Typically sporadic, without known genetic cause or family history.

Symptoms of Wilms tumour include:

• Abdominal Swelling or Mass : Commonly noticed as a painless lump or asymmetrical abdomen.
• Abdominal Pain : Discomfort due to pressure from the tumor.
• Hematuria : Blood in urine, occasionally indicating Wilms tumor.
• High Blood Pressure (Hypertension) : Sometimes detected during routine check-ups.
• Fever : Occasionally present in children with Wilms tumor.
• Loss of Appetite and Weight Loss : Often accompanied by unintentional weight loss.
• Fatigue : General tiredness, a nonspecific symptom.
• 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 : Specific gene mutations in kidney development and cell growth increase Wilms tumor risk, often occurring spontaneously.
• Genetic Syndromes : Syndromes like Beckwith-Wiedemann, WAGR (Wilms tumor, Aniridia, Genitourinary anomalies, intellectual Retardation), and Denys-Drash increase Wilms tumor risk.
• Family History : While most cases are sporadic, a small percentage have familial predisposition.
• Kidney Development : Wilms tumor arises from abnormal fetal kidney cell development.
• Environmental Factors : No definitive environmental links, but prenatal exposure to certain substances may influence risk.

Wilms tumour Risk factors

• Age : Most common in children aged 3-4 years, rare in older children and adults.
• Genetic Syndromes : Linked to higher Wilms tumor risk:
  • Beckwith-Wiedemann syndrome : Causes overgrowth and increases cancer risk, including Wilms tumor.
  • WAGR syndrome : Increases Wilms tumor risk.
  • Denys-Drash syndrome : Rare disorder with kidney abnormalities and elevated Wilms tumor risk.
• Family History : History of Wilms tumor or specific genetic syndromes increases risk.
• Gender : More frequent in girls than boys.
• Race and Ethnicity : Some studies suggest higher incidence in African-American children.
• Birth Weight and Size : Large birth weight and certain birth abnormalities may slightly increase Wilms tumor risk.

What are the Complications?

• Metastasis: Wilms tumor can spread to lungs, liver, and lymph nodes, requiring aggressive treatment.
• Tumor Rupture: May cause abdominal pain, swelling, infection, and increase cancer spread risk.
• Hypertension (High Blood Pressure): Can result from tumor affecting blood vessels, requiring careful management.
• Renal Dysfunction: Tumor size and location can impair kidney function or lead to kidney failure.
• Side Effects of Treatment: Includes fatigue, nausea, vomiting, hair loss, and increased infection risk from surgery, chemotherapy, and radiation therapy.

Diagnosis of Wilms tumour:

• Medical History and Physical Examination: Includes symptoms assessment and overall health evaluation to detect signs of kidney tumor.
• Imaging Studies: Abdominal ultrasound is often the initial imaging test to visualize kidneys and masses.
• CT Scan (Computed Tomography): Provides detailed abdominal images to assess tumor size, location, and extent.
• MRI (Magnetic Resonance Imaging): Further evaluates tumor characteristics and relationships with surrounding structures.
• Biopsy: Sometimes performed to confirm tumor type by examining a small tissue sample 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.

Treatment options for Wilms tumour:

• Surgery (Nephrectomy): Primary treatment involves surgically removing the affected kidney, often sparing healthy tissue. Minimally invasive techniques may be used.
• Chemotherapy: Given before and after surgery to shrink the tumor and eliminate remaining cancer cells. Includes drugs like vincristine, dactinomycin, and doxorubicin.
• Radiation Therapy: Used if there's a high recurrence risk or tumor spread to lymph nodes or other areas. Targets and destroys cancer cells with high-energy beams.
• Targeted Therapies: Experimental treatments targeting specific tumor characteristics, often combined with other therapies.
• Clinical Trials: Considered for high-risk cases to access innovative treatments and advance Wilms tumor research.

Wilms tumour Dos and Don’ts