Inherited disorders associated with pancreatic neuroendocrine tumors

Syndrome	Associated clinical features	Chromosomal location	Pancreatic neuroendocrine tumor type
MEN1	Primary hyperparathyroidism Pituitary tumors Less commonly Adrenocortical tumors Carcinoid tumors Nonmedullary thyroid tumors	11q13	Nonfunctional Gastrinoma Insulinoma Various
Von Hippel-Lindau disease (VHL)	Pheochromocytoma (often bilateral) Retinal and cerebellar hemangioblastomas Renal cell carcinoma	3p25-26	Nonfunctional Various, including cystic tumors
Neurofibromatosis 1 (von Recklinghausen disease)	Neurofibromas Café au lait spots Pheochromocytoma	17q11.2
Tuberous sclerosis	Cardiac rhabdomyomas Renal cysts Angiomyolipomas	9q33.34 and 16p13.3

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