They are very rare with an incidence of ~0.000005% or less than 1 case per 20 million. Equal incidence in middle-aged men and women.
Most patients present with a necrolytic migratory rash and various other elements of the 4D syndrome which includes:
- dermatitis / necrotising migratory erythema
- diabetes mellitus
- deep vein thrombosis
Stomatitis, diarrhoea, anaemia, and weight loss may also occur.
Plasma level of glucagon is elevated (>500 pg/mL).
Tumour size is variable, but most are large (>5 cm) and have metastasised at the time of diagnosis. Most are located in the distal pancreas and are vascular.
Tumours may be solid or contain central low-attenuation areas on CT.
Treatment and prognosis
Approximately 50% of patients survive for at least 5 years post-diagnosis.
- cystic neoplasm (cystic pancreatic mass differential diagnosis)
- solid neoplasm
- nonepithelial pancreatic neoplasms
pancreatitis (mnemonic for the causes)
- gallstone pancreatitis
- interstitial oedematous pancreatitis
- necrotising pancreatitis
- haemorrhagic pancreatitis
- revised Atlanta classification of acute pancreatitis
- chronic pancreatitis
- Ascaris-induced pancreatitis
- tropical pancreatitis
- autoimmune pancreatitis
- emphysematous pancreatitis
- hereditary pancreatitis
- pancreatitis associated with cystic fibrosis
- segmental pancreatitis
- acute pancreatitis
- pancreatic atrophy
- pancreatic lipomatosis
- pancreatic trauma