Rare endocrine tumours of the GI tract.

Functional pancreatic endocrine tumors other than gastrinoma and insulinoma are quite rare. The principles of management include the diagnosis and management of the functional hormonal syndrome, and management of the potentially malignant tumor. Optimally, control of the hormonal syndrome is achieved preoperatively to stabilize the patient status for the operation, however, resection may be an important part of the control of the hormonal syndrome. Ultimately, the only curative treatment for these neoplasms is complete tumour resection, when feasible.