• Incidence 1:20 000000
• Alpha cell tumour of pancreas
• Invariably pancreatic primary
• 75% have metastasized at diagnosis
• Rarely found in association with MEN1

Features

• Rash: Necrolytic Erythema Migrans
• Impaired glucose tolerance, Diabetes Mellitus
• Venous thrombosis
• Normochromic normocytic anaemia
• Decreased weight