Prevention

The causes of most NETs are not fully understood, and there is currently no clear prevention strategy. However, when there is a history of hereditary conditions, such as those listed below, the risk is greater, and genetic surveillance and regular reviews need to be in place to detect early asymptomatic cancers.

Risk factors

The risk factors for developing NETs include:

• hereditary conditions such as multiple endocrine neoplasia type 1 (MEN–1), multiple endocrine neoplasia type 2 (MEN-2), von Hippel-Lindau disease (VHL) and phacomatoses (neurocutaneous syndromes)
• genetic disorders associated with multiple tumours (e.g. tuberous sclerosis and neurofibromatosis)
• conditions that affect stomach acid (e.g. pernicious anaemia and chronic atrophic gastritis)
• age – although NETs can occur at any age, there are some age groups that specific NETs can occur in, such as appendiceal NETs in a younger age group.

Early detection

Increased awareness among health professionals is paramount to enable early diagnosis. There is often a prolonged delay in diagnosis because features are non-specific. GPs should have a strong clinical suspicion of patients who present with a combination of symptoms or persistent symptoms listed in Step 2.

People with a family history of hereditary disorders should be referred to a familial cancer service, geneticist or oncologist for genetic screening.

Screening and surveillance recommendations

There is no national screening program for non-hereditary NETs.

Refer to the optimal care pathway for people with NETs for hereditary conditions that may predispose people to forming a NET.

Checklist

• Referral to a familial cancer service, geneticist or oncologist for genetic screening if there is a known history of hereditary NETs
• Recent weight changes discussed and the patient’s weight recorded
• Alcohol intake discussed and recorded and support for reducing alcohol consumption offered if appropriate
• Smoking status discussed and recorded and brief smoking cessation advice offered to smokers
• Physical activity recorded
• Referral to a dietitian considered
• Referral to a physiotherapist or exercise physiologist considered
• Education on being sun smart considered