Insulinomas tend to be small lesions. A full 40% are 1 cm or less in diameter at the time of operation, with 66% smaller than 1.5 cm and 90% less than 2 cm in maximal diameter.20 These lesions are distributed equally in the head, body, and tail of the pancreas. Ectopic tumors are rare (less than 1%), and when present they are found in close proximity to the pancreas. Malignant insulinomas are rare, accounting for 5% to 10% of cases. Malignancy is defined by evidence of local invasion into surrounding soft tissue or by the presence of distant (lymph node or liver) metastases. Multiple tumors are present in 8% to 17% of cases. When insulinomas are multiple, MEN 1 should be suspected (Fig. 79-6A). Approximately 10% of patients with hyperinsulinism have MEN l.21 Hyperparathyroidism resulting from chief cell hyperplasia (Fig. 79-6B) and pituitary microadenomas resulting in hyperprolactinemia are the most common associated findings in this group of patients.

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