Some people have hereditary conditions that may predispose them to forming a NET. These people should be surveilled for syndrome-specific diagnosis and monitored annually. Hereditary conditions include (Rogel Cancer Centre University of Michigan 2021):

• multiple endocrine neoplasia (MEN)
  • MEN-1 – at risk of developing pituitary adenomas (PitNETs), pancreatic neuroendocrine tumours (pNETs) and hyperparathyroidism
  • MEN type 2A (RET gene mutation) – at risk of developing medullary thyroid cancer, hyperparathyroidism and phaeochromocytoma, noting that some families will only express medullary thyroid cancer
  • MEN type 2B (also known as MEN-3) (RET) – at risk of developing medullary thyroid cancer at a very young age, pheochromocytoma and mucosal neuromas of the lips, tongue and eyelids
  • MEN type 4 (CDKN1B gene mutation) – at risk of developing pituitary tumours, pancreatic NETs and hyperparathyroidism
  • MEN type 5 (MAX) – at risk of developing phaeochromocytomas and pituitary NETs
• VHL– at risk of developing pheochromocytomas, central nervous system and retinal haemangioblastomas, inner ear tumours, kidney tumours and pancreatic NETs
• succinate dehydrogenase gene complex (SDHA/B/C/D) mutations / paraganglioma syndrome – increased risk of phaeochromocytomas, paragangliomas, stomach gastrointestinal stromal tumours, kidney cancers and PitNETs
• fumarate hydratase mutations, which cause hereditary leiomyomatosis and renal cell carcinoma syndrome characterised by renal carcinomas, benign smooth muscle tumours of the skin and uterus and, occasionally, phaeochromocytoma and paragangliomas
• TMEM127 mutations – increased risk of phaeochromocytomas, paragangliomas and renal cell cancers.

For more information visit:

• phaeochromocytoma/paraganglioma panel testing
• VHL disease risk management