1. NEPHROBLASTOMA (WILM’S TUMOR)
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2. EPIDEMIOLOGY WT is a tumor of the developing kidney
WT is the most common renal tumor in children.
Incidence is approximately 0.8 cases per 100,000 persons in the US.
WT formed 15% of all childhood cancers in Enugu (1999 – 2004)
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3. EPIDEMIOLOGY Peak age at diagnosis is between 3yrs and 4 years
Mean age at diagnosis is 3.5years
Most cases (80%) are diagnosed before 5yrs of age
No sex predilection
Most cases are SPORADIC (not related to any hereditary or genetic tendency)
Only about 2.5% is familial
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4. AETIOLOGY The clinical settings include: Sporadic Familial
Associated with genetic syndromes (12-15%)
The familial type is associated with genito-urinary abnormalities like hypospadias, cryptorchidism, ureteral duplication, polycystic kidneys
The genetic syndromes that may be associated with WT:
Beckwith-Wiedemann syndrome
Unilateral hemihypertrophy
Aniridia
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5. PATHOPHYSIOLOGY The aetiology is unknown.
The pathophysiology is characterized by an abnormal proliferation of the metanephric blastema.
Histology shows 3 components: primitive metanephric blastema, dysplatic tubules and the mesenchyme
The degree of differentiation of each component determines whether the tumour has favourable or unfavourable histology.
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6. PATHOPHYSIOLOGY WT can arise anywhere within the kidney
It distorts the renal architecture unlike Neuroblastoma which only displaces the kidneys downward without distorting its architecture
Spreads beyond a tumor capsule into the renal sinus, intra-renal lymphatics and blood vessels → metastases (commonly to the lungs & liver)
Vigorous abdominal palpation spills the cells into the abdominal cavity and encourages dissemination
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7. CLINICAL FEATURES
Asymptomatic flank mass is the most common feature (Crosses the midline in 10% of cases)
Others include:
Abdominal pain in 30-40% of cases.
Gross haematuria in 5-10% of cases.
Constipation from pressure effect.
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8. CLINICAL FEATURES
Severe anaemia with fatigue (secondary to haematuria)
Mild-to-moderate hypertension in 10% of cases.
Fever from tumour necrosis
Cough & dyspnea from pulmonary metastases.
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9. INVESTIGATIONS Ultrasound – initial screening tool
Computed tomography scan
Differential diagnosis of a kidney tumor versus adrenal tumor (neuroblastoma)
Liver metastases
Chest x-ray - As a baseline for pulmonary metastases
Magnetic resonance imaging
Biopsy should be avoided to prevent spillage
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10. CLINICAL STAGING
Stage I: The tumor is limited to the kidney and is excised completely.
Stage II: The tumor extends beyond the kidney into peri-renal tissue but is excised completely.
Stage III: Residual intra-abdominal tumor (nonhematogenous) exists after surgical resection.
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11. CLINICAL STAGING
Stage IV: Hematogenous or lymph node metastasis has occurred outside the abdomen or pelvis.
Stage V: Synchronous bilateral involvement has occurred. Each side is assigned a stage from I to III
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12. MANAGEMNET It is a potentially curable tumor.
Surgical therapy: The first step is surgical staging followed by radical nephrectomy, if possible.
If the tumor is unresectable, biopsies are performed and the nephrectomy is deferred until after chemotherapy, which will shrinks the tumor in most cases.
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13. MANAGEMENT Adjuvant chemotherapy has improved disease-free survival.
The cytotoxic agents are Vincristine, Actinomycin-D and Adriamycin
Radiotherapy is only useful if there is residual bulk disease after surgery.
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14. PROGNOSIS
With the advent of multimodal therapy, the prognosis is good.
The overall cure rate approaches 80-85%.
Cases with diffuse anaplasia and stage III or IV that recur in spite of the complex therapy have a bad prognosis.
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