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Types of Pancreatic Endocrine Neoplasms (PEN) and Related Clinical Syndromes

Tumor Name Cell Type Secreted Hormone Syndrome Location
Insulinoma β Insulin Hypoglycemia Islets
Gastrinoma G Gastrin Pancreatic Ulcer Islets, Stomach, Duodenum
Glucagonoma α Glucagon Diabetes, Necrolytic Migratory Erythema Islets, occasionally Lung, Kidney
VIPoma δ1 Vasoactive Intestinal Peptide Pancreatic Diarrhea Islets, Neuroblastoma
Somatostatinoma δ Somatostatin Inhibition Syndrome Islets, Small Intestine
PPoma PP Pancreatic Polypeptide Asymptomatic or Diarrhea Islets
Neurotensinoma NT Neurotensin Hypotension, Vasodilation, etc. Pancreatic Sympathetic Chain
Carcinoid EC 5-Hydroxytryptamine Carcinoid Syndrome Islets, Digestive Tract

Notes

There are many cells in the islets that have the function of secreting different hormones. Tumors developed from these cells are called pancreatic endocrine neoplasms (PEN). PENs are divided into two categories: one is functional tumors, named according to the main hormone they secrete (as shown in the table above); the other is non-functional islet cell tumors, which have normal serum hormone levels and no clinical symptoms.

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