Glucagon-producing tumors and glucagonoma syndrome

Although glucagon-producing tumors are rare, they are of special clinical interest because patients with these tumors present with characteristic manifestations of glucagonoma syndrome. The features of this syndrome include the typical rash of necrolytic migratory erythema, diabetes, weight loss, anemia, and hypoaminoacidemia. Almost all glucagon-producing tumors arise from alpha cells of the pancreas. The majority of these tumors are malignant and usually patients present with liver metastases. In malignant glucagon-producing tumors, other hormonal syndromes may be present initially or may develop later in the course of the disease. Hyperglucagonemia can also occur in the absence of necrolytic migratory anemia or diabetes. Surgical cure is occasionally possible when the tumor is benign or if a malignant tumor is detected early. Characteristically, malignant tumors and metastases grow slowly. Thus, palliative therapies, e.g., surgical debulking, hepatic artery embolization, partial hepatectomy, octreotide therapy, and chemotherapy, significantly add to the length and quality of life.