Pancreatic endocrine tumors arise from islet and gastrin-producing cells and often produce many hormones. Although these tumors develop most often in the pancreas, they may appear in other organs, particularly the duodenum, jejunum, and lung.
These tumors have two general manifestations:
Functioning
Nonfunctioning
Nonfunctioning tumors may cause obstructive symptoms of the biliary tract or duodenum, bleeding into the gastrointestinal tract, or abdominal masses.
Functioning tumors hypersecrete a particular hormone, causing various syndromes (see table Pancreatic Endocrine Tumors). These clinical syndromes can also occur in multiple endocrine neoplasia, in which tumors or hyperplasia affects two or more endocrine glands, usually the parathyroid, pituitary, thyroid, or adrenals.
Pancreatic Endocrine Tumors
Tumor | Hormone | Tumor Location | Symptoms and Signs |
|---|---|---|---|
ACTHoma | ACTH | Pancreas | |
Gastrin | Pancreas (60%) Duodenum (30%) Other (10%) | Abdominal pain, peptic ulcer, diarrhea | |
Glucagon | Pancreas | Glucose intolerance, rash, weight loss, anemia | |
GRFoma | Growth hormone releasing factor | Lung (54%) Pancreas (30%) Jejunum (7%) Other (13%) | |
Insulin | Pancreas | Fasting hypoglycemia | |
Somatostatinoma | Somatostatin | Pancreas (56%) Duodenum/jejunum (44%) | Glucose intolerance, diarrhea, gallstones |
Vasoactive intestinal peptidase | Pancreas (90%) Other (10%) | Severe watery diarrhea, hypokalemia, flushing |
Treatment of Pancreatic Endocrine Tumors
Surgical resection
Treatment for functioning and nonfunctioning tumors is surgical resection; however, small (< 2 cm), nonfunctioning tumors can usually be safely observed without surgery.
temozolomide alone (1).
Treatment reference
1. Kunz PL, Graham NT, Catalano PJ, et al: Randomized study of temozolomide or temozolomide and capecitabine in patients with advanced pancreatic neuroendocrine tumors (ECOG-ACRIN E2211). J Clin Oncol 41(7):1359-1369, 2023. doi: 10.1200/JCO.22.01013
