The choice of risk management strategy should take into account current age, other health issues and age-related cancer risk. Risks and benefits of interventions should be discussed with an experienced medical professional.
See Checklist of symptoms and signs to review during follow up consultations.
The impact of lifestyle on cancer risk should be discussed e.g. exercise most days for at least 30 minutes at moderate or strenuous intensity, maintain a healthy weight, have a healthy diet, limit alcohol intake, do not smoke and avoid excessive sun exposure.
Cancer/tumour type |
Recommendations |
|
SDHA^^ |
SDHB |
SDHC |
PGL/PC |
Assessment of symptoms, clinical examination and measurement of blood pressure |
Starting age |
18 years |
5 years |
10 years |
Frequency |
Annual |
Annual |
Annual |
Metanephrines^^^ |
Starting age |
18 years |
5 years |
10 years |
Frequency |
Annual |
Annual |
Annual |
68Ga-Dotatate PET-CT/MRI# |
Starting age/frequency |
Once, with initial imaging (only if surveillance imaging commences at
18 years or older) |
Once, with initial imaging (only if surveillance imaging commences at
18 years or older) |
Once, with initial imaging (only if surveillance imaging commences at
18 years or older) |
MRI base of skull to coccyx |
Starting age |
18 years |
10 years |
18 years |
Frequency |
Once every 5 years |
Once every 2 years |
Once every 4 years |
Renal cancer +
gastric GIST |
MRI to coincide with PGL/PC screening |
Starting age |
18 years |
10 years |
18 years |
Frequency |
Once every 5 years |
Once every 2 years |
Once every 4 years |
Pituitary adenoma |
No evidence of benefit for screening |
^^SDHA pathogenic variants have low penetrance (less than 5%) and the benefits of screening are unclear.
^^^Annual fasting free plasma metanephrines or 24 hour urine fractionated metanephrines, and if available plasma or urine 3-methoxytyramine; measurement of 3-methoxytyramine has higher sensitivity for head and neck tumours.
# If the timing of the PET scan coincides with scheduled MRI base of skull to coccyx, limiting the MRI to regions in which the PET scan may miss small lesions can be considered. This decision should be made in consultation with the relevant imaging service/s.
Screening cessation
For individuals who reach age 70 years, and who have had prior screening with no SDH-related abnormalities detected, the screening frequency can be reduced to once every five years. Screening can be ceased at age 80 years, provided no SDH-related abnormalities have been detected. For individuals over 80 years of age who have had no prior surveillance, one-off surveillance imaging can be considered depending on patient preference, life expectancy and co-morbidities.
Additional information
SDHA pathogenic variants demonstrate low penetrance. Particular care should be taken in formulating follow-up plans for asymptomatic individuals with SDHA pathogenic variants to avoid over-surveillance.