Genetic test in multiple endocrine neoplasia type 1 syndrome: An evolving story

Table 1 Suggested guidelines for biochemical and instrumental surveillance screenings in individuals bearing any MEN1 gene mutation

Tumour/lesion	Recommended age of begin screenings (yr)	Biochemical screenings	Frequency	Instrumental screenings	Frequency
Parathyroid adenoma	8	Fasting total serum calcium concentration (corrected for albumin) and/or ionized-serum calcium concentration	Yearly	None	NA
		Fasting serum concentration of full-length PTH
Gastrinoma	20	Fasting serum gastrin concentration	Yearly	None	NA
Insulinoma	5	Fasting serum glucose and insulin concentration	Yearly	None	NA
Other pancreatic neuroendocrine tumours	Under 10	Serum concentration of chromogranin-A, glucagone, pancreatic polypeptide, vasoactive intestinal peptide	Yearly	Abdominal CT, MRI or EUS	Yearly
Anterior pituitary adenoma	5	Serum concentration of prolactin and insulin-like growth factor-1	Yearly	Head MRI	Every 3-5 yr (depending on results of biochemical screenings)
Adrenal gland tumour	Under 10	None unless symptoms or signs of functioning tumour and/or tumour > 1 cm are recognised by imaging	NA	Abdominal CT or MRI	Yearly with pancreatic imaging
Thymic and bronchial carcinoid	15	None	NA	Thoracic CT or MRI	Every 1-2 yr

CT: Computed tomography; MRI: Magnetic resonance imaging; EUS: Endoscopic ultrasound; NA: Not applicable.