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Referral and warning signs
When to refer
Immediate referral to specialized Paediatric Oncology/Surgery centre for ALL suspected cases.
Red flags
- Palpable abdominal mass in a young child
- Haematuria
- Unexplained hypertension in a child
Signs and symptoms
- Painless abdominal mass or swelling (most common presentation), often discovered incidentally by parent.
- Abdominal pain.
- Haematuria (microscopic or gross).
- Fever.
- Hypertension (due to renin secretion by tumour).
- Anorexia, malaise, weight loss (less common).
- Symptoms of metastatic disease (lungs most common): Cough, dyspnoea.
Risk factors
- Associated with certain congenital syndromes and anomalies: WAGR syndrome (Wilms', Aniridia, Genitourinary anomalies, mental Retardation), Denys-Drash syndrome, Beckwith-Wiedemann syndrome.
- Family history (rare).
Diagnosis
Clinical assessment
Suspect in young child (peak age 2-3 years) presenting with asymptomatic abdominal mass, often unilateral, firm, smooth, rarely crossing midline. Assess blood pressure.
Investigations
- Abdominal Ultrasound: Initial imaging test. Confirms intrarenal origin of mass, assesses contralateral kidney, checks for tumour thrombus in renal vein/IVC.
- CT Scan (Abdomen/Pelvis with contrast) or MRI Scan: Defines tumour extent, relationship to vessels, lymph node involvement, contralateral kidney.
- Chest Imaging (Chest X-ray +/- Chest CT): Assess for pulmonary metastases.
- Urinalysis: Check for haematuria.
- Renal function tests (U&Es, Creatinine).
- Histopathology: Diagnosis usually confirmed after surgical nephrectomy (removal of kidney + tumour). Pre-operative biopsy generally avoided unless unresectable or bilateral disease, due to risk of tumour spillage. Histology determines favourable vs unfavourable type (anaplasia - poorer prognosis).
Diagnostic criteria
Imaging findings highly suggestive of Wilms' tumour (intrarenal mass in child) +/- histological confirmation post-nephrectomy.
Management
Non-pharmacological management
- Referral to specialized Paediatric Oncology/Surgery centre.
- Management based on staging and histology.
- Surgery (Nephrectomy): Radical nephrectomy (removal of kidney, tumour, adrenal gland, surrounding fascia) is primary treatment for unilateral disease. Careful handling to avoid tumour rupture.
- Radiotherapy: Used for higher stage disease, unfavourable histology, or pulmonary metastases.
Pharmacological treatment
- Chemotherapy: Integral part of treatment. Given pre-operatively (in some protocols, esp. Europe/SIOP) or post-operatively (standard in North America/COG). Duration and intensity depend on stage/histology. Common drugs: Vincristine, Actinomycin D, Doxorubicin (for higher risk).
Monitoring
Monitor for treatment toxicity. Surveillance for relapse (imaging - ultrasound, CXR/CT). Long-term monitoring for late effects (renal function in remaining kidney, cardiac effects of Doxorubicin, secondary malignancies).
Complications
- Tumour rupture (intra-operative or spontaneous)
- Hypertension
- Metastases (lung, liver, lymph nodes)
- Complications related to surgery (bleeding, infection)
- Treatment-related toxicity (chemo side effects, radiation effects - scoliosis, impaired growth)
- Chronic kidney disease (if remaining kidney affected)
- Relapse
- Secondary malignancies
Prevention
- Screening (regular ultrasound) for children with known predisposing syndromes (e.g., Beckwith-Wiedemann).
Patient education
- (To parents/guardians) Explain Wilms' tumour as kidney cancer in children.
- Need for specialist care (surgery, chemo +/- radiation).
- Explain diagnostic tests and staging.
- Discuss treatment plan, duration, side effects.
- Importance of follow-up and long-term monitoring.
- Good prognosis for most children with favourable histology.
Uganda-specific notes
One of the common childhood solid tumours. Requires specialized centre (UCI) for appropriate diagnosis, staging, surgery, chemotherapy, and radiotherapy. Outcome dependent on access to timely and complete multimodal therapy.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 12, Section 12.3.1 (Listed as Nephroblastoma)
- Uganda Cancer Institute Treatment Guidelines / Paediatric Oncology Protocols
(Specific protocols)