Oncology - Wilms Tumour

Question 1

Definition

Answer 1

Also known as nephroblastoma = MC malignant tumour in children.
The tumour consists of the blastema (immature kidney mesenchyma), primative glomeruli and tubules, and stromal cells.

Question 2

WAGR syndrome

Answer 2

Wilms tumour
Anirdia
Genitourinary anomalies
mental Retardation

Question 3

Deny’s-Drash syndrome

Answer 3

Associated with the greatest risk of Wilms’ tumour (90%):
- Wilms’ tumour,
- Renal failure
- Ambiguous genitalia

Question 4

Beckwith-Wiedemann syndrome

Answer 4

Congenital overgrowth syndrome associated with:
- Wilms’ tumour,
- Hemihypertrophy,
- Organomegaly,
- Neonatal hypoglycaemia

Question 5

Hemihypertrophy

Answer 5

A genetic disorder characterised by overgrowth of one side of the body

Question 6

Epidemiology

Answer 6

Wilms’ tumour is the most common malignant renal tumour in children.
- Young children : most commonly presents at 2-5 years of age, with a median age of 3 years old
- Family history
- Congenital syndromes : WAGR, Deny-Drash, Beckwith-Wiedemann syndromes and hemihypertrophy

Question 7

Signs

Answer 7

Abdominal mass: the most common presenting feature
- Condition unilateral in 90% of cases, although metastases are found in 20% of cases (MC in lungs)
Microscopic haematuria
Hypertension

Question 8

Symptoms

Answer 8

Abdominal distention
Flank pain
Systemic symptoms e.g. fever, weight loss (anorexia)
Painless haematuria: macroscopic haematuria is uncommon

Question 9

Diagnosis

Answer 9

FIRST LINE:
- Urinalysis: microscopic haematuria
- U+Es: elevated urea and creatinine, indicating renal dysfunction
- Abdnominal USS: FIRST LINE IMAGING CHOICE = mass arising from kidneys
- Staging CT chest, abdomen, and pelvis: For staging

Question 10

Treatment

Answer 10

All patients with an enlarged, unexplained abdominal mass should be referred for an urgent paediatric review within 48 hours for suspected Wilms’ tumour:
- Radical nephrectomy
- Adjuvant chemotherapy: surgery is often combined with adjuvant chemotherapy
- Adjuvant radiotherapy : offered to patients with more advanced disease
- Renal transplantation : usually reserved for patients with advanced, bilateral disease and renal failure.

Question 11

Complications

Answer 11

Renal failure: rare but occasionally seen in those with bilateral disease
Chemotherapy-related complications: such as bone marrow suppression and neutropenic sepsis