Nephroblastoma Flashcards
What is another name for nephroblastoma?
Wilms tumour
What percentage of childhood cancer is nephroblastoma?
5%
Describe the location of nephroblastoma
Usually unilateral but may be bilateral
Where do many nephroblastoma’s arise from?
Nephrogenic rests
What are nephrogenic rests?
Embryological remnants seen in 1% of infants at birth
What genes are commonly mutated in nephroblastoma
Common tumour suppressor gebes
Name three genetic or overgrowth syndromes associated with nephroblastoma
WAGR
Denys-Drash
Beckwith-Wiedemann
Describe WAGR syndrome
Wilms tumour
Aniridia
Genitourinary malformations
Retardation
Describe the features of nephroblastoma from the history
Abdominal mass Abdominal swelling Fever Haematuria Abdominal pain
Describe the features of nephroblastoma on clinical examination
Distension with unilateral/bilateral palpable renal masses
Hypertension
Compression of intra-abdominal structures
What investigations are useful for nephroblastoma?
FBC U&Es Urine dip - haematuria Renal USS CT/MRI - staging
Describe the staging of nephroblastoma
1 - tumour condined to kidney
2- Tumour has begun to spread beyond the kidney
3- Tumour cannot be completely surgically resected because it has spread to neighbouring lymph nodes or ruptured before/during surgery
4- Distant metastases - most commonly the lungs
5- Bilateral tumours
Describe the initial management of nephroblastoma
Supportive care - treat any co-existing infection and ensure nutrition and hydration are optimised
Describe the definitive and long term management of nephroblastoma
Stage 1 and 2 - surgery (nephrectomy)
Chemo is used neo-adjuvantly or adjuvantly
What is the aim of surgery?
Preserve renal function while removing the malignant tissue
