Clinical Manifestation of Endocrine Tumors of the Pancreas

Abstract

Pancreatic neuroendocrine neoplasms are rare and present with symptoms and signs related to the secreted endocrine hormones. Some neoplasms may secrete more than one hormone. Several of the clinical manifestations of these hormones are specific, but others are not specific and thus the diagnosis may be delayed or overlooked. Insulinomas present with hypoglycemia and require the demonstration of venous hypoglycemia with inappropriate hyperinsulinemia for diagnosis. Gastrinomas present with the consequences of severe gastric hyperacidity, hypergastrinemia, and diarrhea. Glucagonomas present with the classic skin changes of necrolytic migratory erythema and with weight loss. The other pancreatic neuroendocrine neoplasms have less-specific presentations, and often diarrhea is a common feature. VIPomas present with watery diarrhea and hypokalemia. Somatostatinomas present with steatorrhea, hyperglycemia, and gallstones. Patients presenting with classic Cushing's syndrome from ectopic ACTH and with the classic carcinoid syndrome may rarely have pancreatic tumors eventually identified as the cause. Specialized endocrine investigations are necessary if such tumors are suspected, and the prognosis may be better with earlier diagnosis and treatment.