Wilms tumour Flashcards
1
Q
Wilms tumour is what type of cancer?
A
nephroblastoma
2
Q
Average age of patients with Wilms tunmour
A
children<5yrs
median age of 3 years old.
3
Q
Conditions associated with Wilms tumour development
A
- Beckwith-Wiedemann syndrome
- WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation
- hemihypertrophy
4
Q
Genetic mutation association with Wilms tumour
A
loss-of-function mutation in the WT1 gene on chromosome 11
5
Q
Clinical features of Wilms tumour
A
abdominal mass
painless haematuria
flank pain
other: anorexia, fever
6
Q
What percentage of patients have metastases at presentation of wilms tumour? Where are they most commonly found?
A
20%
Usually lung mets
7
Q
Management of wilms tumour
A
nephrectomy
chemotherapy
radiotherapy if advanced disease
8
Q
Prognosis
A
good, 80% cure rate
9
Q
Hisotological appearance of wilms tumour
A
- epithelial tubules
- areas of necrosis
- immature glomerular structures
- stroma with spindle cells and small cell blastomatous tissues resembling the metanephric blastema
10
Q
A
