The hereditary forms of pancreatic neuroendocrine tumors.

Pancreatic endocrine tumors occur sporadically or in the setting of multiple endocrine neoplasia type I (MEN-1) or von-Hippel-Lindau disease. In the latter circumstances, the tumors are often multiple. This commentary addresses the differences in clinical, pathologic, and molecular features of MEN-1 and von Hippel-Lindau disease associated pancreatic endocrine tumors.