- Very rare (<100 ever reported)
- Syndrome
- Steatorrhoea
- Diabetes Mellitus
- Hypochlorhydria
- Gallstones (from reduced gallbladder emptying)
- Diagnosis rarely made pre-op (as symptoms so non-specific)
- If suspected do fasting somatostatin levels
- Usually solitary, > 2cm
- 60% found in pancreas, usually HOP
- 90% malignant, mets common at diagnosis
- Rarely associated with MEN1
- But can be associated with von Recklinghausen disease and Pheochromocytoma