Wilms tumour (nephroblastoma)

origin= embryonal renal tissue
commonest renal tumour of childhood
> 80% pts present before 5 years old
1. rarely seen after 10 years old
clinical features
1. presentation
  1. common
    1. abdo mass
      1. large
      2. incidental finding in otherwise well child
  2. uncommon
    1. abdo pain
    2. anorexia
    3. anaemia
      1. haemorrhage into mass
    4. haematuria
    5. hypertension
Ix
1. US +/- CT or MRI
  1. showing intrinsic renal mass distorting normal struc
    1. envelopes a remnant of normal renal tissue
  2. characteristic mixed densities visible
    1. cystic and solid
2. staging to assess
  1. for distant mets
    1. usually in lung
  2. initial tumour resectability
  3. function of contralat kidney
Mx
1. initial chemo fb delayed nephrectomy
  1. then tumour histologically staged
    1. then Rx planned
2. radiotherapy
  1. only for those w/ more advanced disease
good prognosis
1. > 80% of pts cured
2. cure rate for pts w/ mets @ presentation is > 60%
relapse has poor prognosis

Resource summary

	Created by v.djabatey about 10 years ago