Somatostatinoma: atypical presentation of a rare pancreatic tumor.

Somatostatinomas are rare neuroendocrine tumors that can result in a variety of symptoms depending on the secretion of other peptides in association with or in response to somatostatin. The rarity and variable clinical presentation of these tumors present problems in diagnosis and management. This report details the treatment of a 66-yr-old male who had a somatostatinoma with an atypical location and presentation. His clinical course was one of recurrent disease treated surgically and the interval development of cholelithiasis. He has survived 5 yr with his tumor, illustrating that monitoring peptide levels and an aggressive surgical approach are warranted for this condition. Prophylactic cholecystectomy should be considered at the time of exploration.