Pancreatic neuroendocrine tumors

Abstract

Pancreatic neuroendocrine tumors (pNETs) are rare neoplasms, with a prevalence of 1–2 per 100,000 people. Based on the presence or absence of a specific hormone-related clinical syndrome, they are divided into functioning and nonfunctioning. Among the former tumors, insulinomas are the most common. pNETs may be sporadic or associated with hereditary syndromes. The histopathology evaluation should include immunostaining with general (chromogranin A and synaptophysin) and specific neuroendocrine markers, as well as with the proliferation index Ki67. Chromogranin A is currently the most useful neuroendocrine biomarker for diagnosis and follow-up of pNETs. Other specific hormones released by the neoplastic cells can also be included in the biochemical evaluation. For tumor localization both noninvasive and invasive techniques may be used. Debulking procedures and medical therapy are the possible treatment options for pNETs, but surgery is the only modality that offers the possibility of cure.