Neural crest tumours – cells that migrate to form sympathetic chain, including adrenal glands.
Usually young children, usually already metastases at presentation – that’s because mostly vague symptoms until an abdominal mass or lymphadenopathy obvious.
Several eye related symptoms possible –
- Dancing eyes (opsoclonus-myoclonus) a famous association – only seen in 1% of neuroblastoma but 1/3 of opsoclonus-myclonus syndrome (includes ataxia too!) have it.
- Horner’s syndrome associated, as sympathetics (dilated pupil) run with oculomotor nerve.
- “Panda eyes” are a rare clinical finding – proptosis, bruising – from orbital mets.
Catecholamines are a marker but only rarely do you get symptoms eg hypertension, sweating, diarrhoea.
Bone pain and fever are not uncommon. Otherwise depends were the mass effects are eg obstructive jaundice, dysphagia.
GD-2 marker. Catecholamines as above.
MRI full body else MIBG scintigraphy.
“Metastatic special” risk category – under 18 months, only skin, liver, marrow. Resolve spontaneously even when extensive!
Screening programmes in Germany and US doubled pick up rate but no change in mortality… Probably because detected more of these Metastatic special cases.