Glucagonoma syndrome: survival 24 years following diagnosis

The symptoms of necrolytic migratory erythema, diabetes, stomatitis, weight loss and diarrhoea represent the glucagonoma syndrome which has been recognized since the early 1970s. Because of its rarity (1 case/20-200 million population) late diagnosis is frequent which leads to a poor prognosis. The case described, originally reported in 1974, is the longest survivor to be documented in the literature, and is one of the original patients with a glucagonoma that helped to define the syndrome.