Clinical features

The presentation depends upon the site of the primary tumour and the extent of metastases; the symptoms can be very varied. Half of cases present with advanced disease. Local infiltration, lymphatic spread, and haematogenous spread, particularly to liver, lungs and bone, is common.

General effects of malignancy:

• pallor - anaemia - fever, weight loss, anorexia, irritability, failure to thrive

Local tumour effects:

• an abdominal mass is common - it may cross the midline and be difficult to distinguish from the liver
• local invasion may cause the cauda equina syndrome, or paraplegia

Metastatic spread:

• proptosis
• Horner's syndrome
• dysphagia
• lung collapse
• bone pain and pathological fracture
• periorbital bruising looking like a black eye is seen in children

Skin involvement:

• skin nodules which are firm and are said to have a blueberry muffin appearance

Paraneoplastic effects:

• flushing, cerebellar ataxia, diarrhoea
• in contrast to phaeochromocytomas, hypertension is rare