Medical therapy of pancreatic neuroendocrine neoplasms

Abstract

Pancreatic neuroendocrine neoplasms (PNENs) are most commonly advanced, low/intermediate-grade of malignancy, nonfunctioning, and somatostatin receptor expressing. However in a small percentage of cases, they are high-grade of malignancy, and prognosis is poor independently from therapy. Medical therapy of PNENs is usually considered in advanced stage of disease. It should be based on the right diagnosis, prognostic estimate, and morphological and functional characterization of disease. Several different options can be considered, including chemotherapy, somatostatin analogs, interferon, molecular-targeted agents, peptide receptor radionuclide therapy, and liver-directed treatments. While in high-grade PNENs, chemotherapy represents the only therapeutic option; in low-/intermediate-grade PNENs, several options should be discussed within a multidisciplinary team. No studies defining sequence, timing, and integration of the different therapies exist so far; therefore, a specific therapeutic strategy for the single patient should be discussed and shared by means of an interactive interdisciplinary discussion, preferably involving a referral center for NEN.