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Table 2 Surveillance strategies proposed by guidelines or expert statements

From: Current prospects of hereditary adrenal tumors: towards better clinical management

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]

  1. 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