Wilms tumour and associated syndromes
Posted by admin
on June 24, 2011
Wilms tumour
- Also known as nephroblastoma.
- Wilms tumour is the most common primary renal tumour in children.
- Usually wilms tumour is a solitary,well circumscribed lesion,but 10% is bilateral or multicentric.
- Triphasic combination of epithelial,stromal and blastemal elements.
- 5% show anaplasia associated with underlying P53 mutation and unresponsive to chemotherapy.
- Nephrogenic cell rests- High correlation with Wilms tumour in contralateral kidney.
Syndromes associated with wilms tumour
WAGR syndrome
- Wilms tumour
- Aniridia
- Genital abnormalities
- Mental retardation
Denys Drash Syndrome
- Gonadal dysgenesis(Male pseudohermaphroditism)
- Renal abnormalities
Beckwith Weidman syndrome
- Organomegaly
- Hemihypertrophy
- Renal medullary cysts
- Hepatoblastoma
- Adrenal cortical tumour
- Rhabdomyosarcoma
- Pancreatic tumour
Like this:
Like Loading...
0 Comments.