In general, treatment for<unk>  encompasses the same array of options as other neuroendocrine tumors, as discussed in that main article. However, there are some specific differences, which are discussed here. In functioning<unk> s, octreotide is usually recommended prior to biopsy or surgery but is generally avoided in insulinomas to avoid profound hypoglycemia.<unk> s in Multiple endocrine neoplasia type 1 are often multiple, and thus require different treatment and surveillance strategies. Some<unk> s are more responsive to chemotherapy than are gastroenteric carcinoid tumors. Several agents have shown activity. In well differentiated<unk> s, chemotherapy is generally reserved for when there are no other treatment options. Combinations of several medicines have been used, such as doxorubicin with streptozocin and fluorouracil (5-FU) and capecitabine with temozolomide. Although marginally effective in well-differentiated PETs, cisplatin with etoposide has some activity in poorly differentiated neuroendocrine cancers (PDNECs), particularly if the PDNEC has an extremely high Ki-67 score of over 50%. Several targeted therapy agents have been approved in<unk> s by the FDA based on improved progression-free survival (PFS): * everolimus (Afinitor) is labeled for treatment of progressive neuroendocrine tumors of pancreatic origin in patients with unresectable, locally advanced or metastatic disease. The safety and effectiveness of everolimus in carcinoid tumors have not been established. * sunitinib (Sutent) is labeled for treatment of progressive, well-differentiated<unk> s in patients with unresectable locally advanced or metastatic disease. Sutent also has approval from the European Commission for the treatment of 'unresectable or metastatic, well-differentiated<unk> s with disease progression in adults'. A phase III study of sunitinib treatment in well differentiated<unk>  that had worsened within the past 12 months (either advanced or metastatic disease) showed that sunitinib treatment improved progression-free survival (11.4 months vs. 5.5 months), overall survival, and the objective response rate (9.3% vs. 0.0%) when compared with placebo.
Pancreatic neuroendocrine tumor
Small intestine neuroendocrine tumor
Metastatic insulinoma
Acinar cell carcinoma of the pancreas
Glucagonoma
Pancreatic mucinous cystic neoplasm
Insulinoma
Goblet cell carcinoid