Neuroendocrine Tumors

Neuroendocrine neoplasms (NEN), also known as neuroendocrine tumors (NET), a tumor arising from the endocrine system, accounts for 0.5 percent of all cancers. Not only are these tumors rare, but they also vary significantly in symptoms, involvement of organ system(s), and prognosis. Therefore, treatment decisions can be challenging. Treatment of these tumors includes symptom management, based on the tumor’s hormone secretion, and direct tumor control. A mainstay of treatment for most NEN is somatostatin analogs (SSA), octreotide and lanreotide. Unfortunately, there is scant literature comparing these treatment options. Therefore, multiple patient factors need to be considered in deciding between these two products. Like other slow-growing cancers, patients diagnosed with metastatic NEN will undergo multiple treatments throughout their disease. Lutetium Lu-177 dotatate, a peptide receptor radionuclide, is used in patients with previously treated somatostatin-positive disease. Data is emerging that this treatment option could be repeated in those who had a previous benefit. Neuroendocrine carcinomas (NECs), a very aggressive form of NENs, have an extremely poor prognosis. Per the NCCN and ENETS guidelines, chemotherapy-based treatment is recommended, but few studies exist regarding the specific medications to utilize in this patient population. Therefore, multiple factors need to be considered when formulating a treatment plan. Lastly, recent and ongoing clinical trials will be discussed related to the treatment of NEN and NEC, focusing on immunotherapy and oral small molecule inhibitors.