Endocrine Tumors of the Pancreas

MICHAEL J. DEMEURE, MD

Neuroendocrine tumors of the pancreas arise from the islet cells within the pancreas. These islands of cells were initially described by Langerhans in 1869 and comprise 1 to 2% of the pancreatic mass.1 The initial report of an islet cell neoplasm was in 1902 by Nichols.2 Approximately 95% of islet cell tumors are insulinomas, gastrinomas, or nonfunctional tumors producing no overt clinical syndrome. The remaining small proportion of these tumors may produce glucagon, vasoactive intestinal peptide (VIP), somatostatin, or other pancreatic peptides. The diagnosis of an islet cell tumor is established based on a clinical suspicion of endocrine hormone excess or by a nonsecretory tumor producing symptoms owing to the size or location of the tumor. Once the diagnosis is established by appropriate biochemical tests, judicious use of a variety of available localizing radio-logic tests is warranted prior to a decision regarding treatment. These radiologic tests will be discussed in general after a presentation of the clinical features of the various islet cell tumors. Their treatment will also be discussed. The selection of a specific operation depends on a variety of factors including pathophys-iology and location of the tumor entity, whether the tumor is benign or malignant, and whether it is a sporadic tumor or is occurring as part of multiple endocrine neoplasia (MEN) type I.

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