Neuroendocrine tumor of the pancreas with glucagon production

Background: Glucagonoma is a rare pancreatic tumor of islet alpha 2 cells. Fewer than 200 cases have been reported worldwide, with an estimated incide nce of 1 in 20 million. In general, the disease is characterized by a well- defined clinical syndrome which typically shows as necrotic migratory eryth ema of the skin, weight loss, diabetes mellitus, anemia, cheilosis and stom atitis. Since pancreatic glucagonomas are predominantly located in the tail and findings of radiographic or sonographic examination can remain unspeci fic, patients often present already metastasis when diagnosis is first esta blished. Case Report: We report the case of a 67-year-old mail with an extended mali gnant glucagonoma of the pancreas infiltrating already the hilus of the spl een and, additionally, presenting metastatic lesions in the liver and the l eft adrenal gland. A monohormonal expression of glucagon could be ascertain ed by serological and immunohistochemical analysis. The special feature of this case is that the tumor was not associated with the characteristic skin rash. Conclusion: An unclear migratory erythema combined with diabetes mellitus a nd stomatitis/cheilosis should lead to the differential diagnosis of glucag onoma. Isolated glucagonomas are very difficult to find out and often diagn osed already presenting metastasis.