Islet cell tumors of the pancreas: clinical, radiologic, and pathologic correlation in diagnosis and localization

Islet cell tumors are rare pancreatic or peripancreatic neoplasms that produce and secrete hormones to a variable degree. These tumors are best divided on clinical grounds into those that produce a recognizable, clinically evident endocrine syndrome (ie, functioning) and those that exhibit no clinical evidence of hormone production (ie, clinically silent). Clinically silent tumors produce symptoms due to mass effect because of their large size. They are often partially cystic or necrotic. Functioning islet cell tumors usually manifest earlier in the course of the disease because of the distinctive signs and symptoms of the associated endocrine syndrome. Clinically silent and functioning tumors cannot be histologically distinguished reliably even with the use of immunohistochemical stains. Insulinoma and gastrinoma, the two most common functioning lesions, are typically small homogeneous masses. Other functioning islet cell tumors include glucagonoma, somatostatinoma, vipoma, and adrenocorticotropic hormone-producing tumor. Larger tumors are associated with calcification, cystic degeneration and necrosis, and a more aggressive behavior (local and vascular invasion as well as distant metastases). There are many different techniques for detection and characterization of these lesions that are usually chosen according to the radiologist's experience and preference. Treatment and prognosis of these lesions depend on the hormone produced, their size, and their behavior.