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Overview of Pancreatic Endocrine Tumors

By

Anthony Villano

, MD, Fox Chase Cancer Center

Reviewed/Revised Oct 2023
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Topic Resources

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 ). 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.

Table

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%)

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.

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 ).

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

Drugs Mentioned In This Article

Drug Name Select Trade
Bynfezia, Mycapssa, Sandostatin, Sandostatin LAR
Somatuline Depot
Xeloda
TEMODAR
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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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