FRCR Paediatrics : Syndromes associated with Wilms tumour

Below is a list of syndromes associated with Wilms tumours.

Hutchinson syndrome  

This is a set of symptoms that is secondary to extensive bone metastases ( in particular, the skull) 

CT head : 

1) Sutural diastasis secondary to dural metastases 

2) Hair on-end appearance of the skull 

3) Proptosis  

Pepper syndrome 

This refers to massive hepatomegaly secondary to extensive hepatic metastases.  

Opsomyoclonus ( dancing eyes-dancing feet syndrome) 

Patients present with jerky eye movements (dancing eyes), myoclonic jerks and cerebellar ataxia 

50% of patients with opsomyoclonus have a neuroblastoma 

Symptoms include:  

•  Opsoclonus ( jerky eye movements - dancing eyes)  
• Myoclonic jerks 
• Cerebellar ataxia
• Behav...