Endocrine tumours of the pancreas

Abstract

Endocrine tumours of the pancreas, also known as islet cell tumours, are neuroendocrine tumours arising from the pancreatic islets. Pancreatic endocrine tumours are uncommon and no endocrine surgeon has vast experience with them. Patients with these rare tumours present either with a specific syndrome or with symptoms related to the malignant nature of the tumours. In addition, an increasing number of 'pancreatic incidentalomas' with no obvious function have been detected by abdominal computerized tomography. Diagnosis relies on a high index of clinical suspicion and is based on the demonstration of elevated serum levels of the secretory products concerned. Therapeutic strategy depends on the exact localization of the primary tumor and the presence or absence of metastasis, and is designed to control the tumoral process as well as ameliorate the syndrome associated with it. The present review highlights the clinical presentation, diagnosis, localization and therapeutic principles in the management of pancreatic endocrine tumours.