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| Islet Cell Neoplasms |
| Etiology • Unknown. |
| Pathogenesis • May be associated with mutation in 11q11-q13 in patients with multiple endocrine neoplasia type I (Werner's syndrome) comprised of parathyroid hyperplasia or adenoma, pituitary adenoma and islet cell adenoma usually (occasionally carcinoma), |
| Epidemiology • Rare • Most common in adults • Can be seen throughout the pancreas or in the case of gastrinomas in duodenum • Single or multiple • Benign or malignant |
| General Gross Description • Well demarcated, yellow to tan, firm nodules |
| General Microscopic Description • Cells are arranged in cords or trabeculae of cells with bland round to oval nuclei with small nucleoli and modest amounts of cytoplasm • Resemble normal islet cells • May see atypia and mitoses which do not indicate malignancy • Malignancy defined by invasion or metastases |
| Clinical Correlation • Symptoms dependent on whether the neoplasm is secreting or non-secreting • Insulinomas lead to hypoglycemia with neurologic symptoms relieved by glucose • Gastrinomas lead to hypersecretion of gastric acid and ulcers (Zollinger-Ellison syndrome) • Most insulinomas (>90%) are benign; only 40% of gastrinomas are benign • Other neoplasms include glucagonomas, somatostatinomas and VIPomas |
| References • Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 1169-70; 922-24. |
| Islet Cell Neoplasms |
| Synopsis by: Melinda Sanders M.D. (T59000M81503)[408] |
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