These tumors have two general forms:
Endocrine pancreatic tumors originate from the island and gastrin cells and often secrete many hormones. Although these tumors usually develop in the pancreas, they may also in other organs, particularly the duodenum, jejunum and in the lungs, occur. These tumors have two general forms: Functionally active Functionally inactive Functionally inactive tumors can cause obstructive symptoms of biliary tract or the duodenum and cause bleeding or abdominal masses gastrointestinal. Functionally active tumors hyper secrete a specific hormone, causing various syndromes (s. Endocrine pancreatic tumors). These clinical syndromes may also occur at a multiple endocrine neoplasia, in which two or more endocrine gland tumors or hyperplasia, usually the parathyroid, pituitary, thyroid or adrenal concerning (overview of multiple endocrine neoplasia). Endocrine pancreatic tumors tumor hormone tumor localization symptoms and complaints ACTHom ACTH pancreatic gastrinoma Cushing’s syndrome (gastrinoma) gastrin pancreas (60%) of the duodenum (30%) Other (10%) abdominal pain, peptic ulcers, diarrhea glucagonoma (glucagonoma) Glucagon pancreatic glucose intolerance, rash, weight loss, anemia GRFom somatotropin (growth hormones-) releasing factor lung (54%) pancreatic (30%) jejunum (7%) Other (13%) acromegaly insulinoma (insulinoma) Insulin pancreatic Nüchternhypoglykämie Somatostatinoma somatostatin pancreatic (56%) of the duodenum / jejunum (44%) Glucose intolerance, diarrhea, gallstones VIPoma (VIPoma) Vasoactive intestinal polypeptide pancreatic (90%) Others (10%) Heavy aqueous diarrhea, hypokalemia, paroxysmal flushing (flushing symptoms) The therapy for functionally active and inactive tumors is surgical resection. If metastases do not allow curative surgery, (e.g. octreotide, lanreotide) may be attempted in various tumors functionally active anti-hormonal therapies. Because of the rarity of these tumors chemotherapy studies were able to identify any definitive therapies. Streptozotocin has selective activity against pancreatic islet cells and is commonly used alone or in combination with 5-fluorouracil or doxorubicin. Some centers use chlorozotocin and interferon. Newer chemotherapy regimen containing temozolomide either alone or in combination with other substances (for example, thalidomide, bevacizumab, everolimus, capecitabine) showed promising results in small clinical trials and are currently being investigated in large prospective clinical trials.