Table 3. Genetic syndromes associated with pancreatic endocrine neoplasm (PEN).

Syndrome	Gene location	Gene product	PEN frequency and tumour type
MEN-1	11q3	Menin	80–100% (non-functioning, gastrinoma, insulinoma)
VHL	3p25.5	pVHL	12–17% (all non-functioning)
NF-1	17q11.2	Neurofibromin	6% somatostatinoma
TSC	9q34 (TSC1) 16p13.3 (TSC2)	Hamartin, tuberin	<5% either functioning or non-functioning

MEN-1, multiple endocrine neoplasia-1; NF-1, neurofibromatoses-1 (von Recklinghausen disease); TSC, tuberous sclerosis complex; VHL, von Hippel–Landau.