Of all the 43 patients, we reported only one PNEN-related death (2.3%), occurring at 61 years of age for multiple liver metastases from gastrinoma, diagnosed two years after the initial PD in which we had found and removed one duodenal gastrinoma, one gastrin-secreting lymph node metastasis, one gastrin-secreting liver metastasis, and nine NF-PNENs in the pancreas head. The gene discussed is GAST; the disease is gastrin-producing neuroendocrine tumor.