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:
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 ). These clinical syndromes can also occur in multiple endocrine neoplasia Overview of Multiple Endocrine Neoplasias (MEN) The multiple endocrine neoplasia (MEN) syndromes comprise 4 genetically distinct familial diseases involving adenomatous hyperplasia and malignant tumors in several endocrine glands. MEN 1 involves... read more , in which tumors or hyperplasia affects two or more endocrine glands, usually the parathyroid, pituitary, thyroid, or adrenals.
Pancreatic Endocrine Tumors
Symptoms and Signs
Abdominal pain, peptic ulcer, diarrhea
Glucose intolerance, rash, weight loss, anemia
Growth hormone releasing factor
Glucose intolerance, diarrhea, gallstones
Vasoactive intestinal peptidase
Treatment of Pancreatic Endocrine Tumors
Treatment for functioning and nonfunctioning tumors is surgical resection; however, small (< 2 cm), nonfunctioning tumors can usually be safely observed without surgery.
If metastases preclude curative surgery, various antihormone treatments (eg, octreotide, lanreotide) may be tried for functioning tumors.
Because of tumor rarity, few trials exist to guide definitive treatment. One study has shown that, with metastatic disease, the combination of capecitabine and temozolomide improved median progression-free survival from 14.4 months to 22.7 months over temozolomide alone (1 Treatment reference 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... read more ).
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
Drugs Mentioned In This Article
|Drug Name||Select Trade|
|Bynfezia, Mycapssa, Sandostatin, Sandostatin LAR|