Syndrome | Associated clinical features | Chromosomal location | Pancreatic neuroendocrine tumor type |
MEN1 | Primary hyperparathyroidism Pituitary tumors Less commonly
| 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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