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Overview of Pancreatic Endocrine Tumors

By

Minhhuyen Nguyen

, MD, Fox Chase Cancer Center, Temple University

Last full review/revision Jun 2019| Content last modified Jun 2019
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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.

Table
icon

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

  • Surgical resection

Treatment for functioning and nonfunctioning tumors is surgical resection. If metastases preclude curative surgery, various antihormone treatments (eg, octreotide, lanreotide) may be tried for functioning tumors. Because of tumor rarity, chemotherapy trials have not yet identified definitive treatment. Streptozotocin has selective activity against pancreatic islet cells and is commonly used, either alone or in combination with 5-fluorouracil or doxorubicin. Some centers use chlorozotocin and interferon.

Newer chemotherapeutic regimens that include temozolomide, either alone or in combination with other agents (eg, thalidomide, bevacizumab, everolimus, capecitabine), have shown good results in small clinical trials and are under active investigation in large prospective clinical trials.

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