3: Haematology - Wilm's Tumour Flashcards
What is wilm’s tumour also referred to as
Nephoblastoma
What is wilm’s tumour
Malignancy of the kidney that occurs during childhood
What age children does Wilms tumour affect
Under 5’s
What is the peak incidence of Wilm’s tumour
3-years
What gives a genetic predisposition to Wilm’s tumour
Mutation in WT1
What 3 syndromes is wilm’s tumour associated with
- WAGR
- Denny-Drash
- Beckwith wiedeman syndrome
What causes WAGR syndrome
Deletion of WT1
Where is WT1 located
Chromosome 11
What is WAGR syndrome
Wilms tumour
Anirida
GU anomalies
mental Retardation
What is aniridia
Absent iris
what GU anomalies can be present in WAGR
- Undescended testes
- Pseudohermaphroditism
What is denny-drash syndrome
Mutation in WT1
How does denny drash syndrome present
- Wilms tumour
- Pseudohermaphroditism or undescended testes
What causes beckwith wiedemann syndrome
Mutation in WT2
What are two features of beckwith weidermann syndrome
Organomegaly
Infantile hypoglycaemia
