| Normal pancreas in this panel is represented by the
section of the far right.
The lobulated appearance is typicla.
The neoplasm is reddish brown in these sections and obliterates the normal architecture. |
| 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 |
| Clinical |
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 For more information on prognosis and treatment, consult the NCI web site. |
| General Gross Description |
Well demarcated, yellow to tan, firm nodules |
| General Micro 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 |
| Reference |
Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 1169-70; 922-24.
|