Table 2.

Surveillance strategies proposed by guidelines or expert statements

Hereditary tumor	Surveillance method of adrenal tumor	Guidelines/ statements	References
LFS	For children: abdominal and pelvic ultrasound, and alternative blood test (total testosterone, dehydroepiandrosterone, and androstenedione) (every 3–4 months)	Japanese LFS medical guidelines; Toronto protocol	[15, 16 ]
LS	NA	NA
FAP	NA	NA
MEN2	Annual measurement of plasma free or 24-hour urine fractionated metanephrine/normetanephrine	NCCN guidelines	[4]
VHL	Plasma-metanephrine/normetanephrine (every year from age 5); abdominal MRI (every 2-years from age 15, no upper limit of age)	VHL Alliance; Danish guidelines	[17, 18]
NF1	Biochemical testing in individual who has raised blood pressure	ERN GENTURIS guidelines	[19]
Hereditary PPGL syndrome	Blood pressure monitoring at all medical visits, annual measurement of plasma free metanephrine or 24-hour urine for fractionated metanephrine, and whole body MRI every 2-3 years (from age 6–10 for patients with SDHB mutations and age 10–15 for patients with all other SDHx mutations)	NCCN guidelines	[4]

LFS Li-Fraumeni syndrome: LS Lynch syndrome: FAP familial adenomatous polyposis: MEN2 multiple endocrine neoplasia type 2: VHL von Hippel-Lindau disease: NF1 Neurofibromatosis type 1: PPGL pheochromocytoma/paraganglioma: NA not available: NCCN National Comprehensive Cancer Network: ERN GENTURIS European Reference Network on Genetic Tumor Risk Syndromes