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Box 94.1

Paragangliomas (Phaeochromocytomas)

  • These are tumours of chromaffin cells, derived from neural crest tissue.
  • Most commonly occur in the adrenal medulla, in which instance they are called a phaeochromocytomas. Often secrete catecholamines (adrenaline, noradrenaline, dopamine) but may secrete other hormones as well.
  • Usually sporadic, but are also associated with several genetic syndromes including multiple endocrine neoplasia (type 2A or 2B), von Hippel-Lindau disease, succinate dehydrogenase (SDH) mutations and neurofibromatosis type 1.
  • Usually present between the ages of 20 and 50 years, with earlier presentation increasing the likelihood that they are due to one or other of the genetic syndromes.