Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • Migratory necrolytic dermatitis, usually involving the legs and perineum• Weight loss• Stomatitis• Thrombophlebitis• Anemia• Mild to moderate diabetes mellitus +++ Epidemiology + • Arise from cells in the pancreatic islets• Most tumors are solitary and large (> 4 cm) located in the body or tail of the pancreas• About 25% are benign and confined to the pancreas-The remainder has metastasized by the time of diagnosis, most often to the liver, lymph nodes, adrenal gland, or vertebrae• The age range is 20-70 years, and the condition is more common in women +++ Symptoms and Signs + • Migratory necrolytic dermatitis, usually involving the legs and perineum• Weight loss• Stomatitis• Thrombophlebitis +++ Laboratory Findings + • Elevated serum glucagon level (> 1000 pg/mL)• Hypoaminoacidemia• Anemia• Hyperglycemia +++ Imaging Findings + • CT scan or MRI demonstrates the tumor and sites of metastases + • The diagnosis may be suspected from the distinctive skin lesion-In fact, the presence of a prominent rash in a patient with diabetes mellitus should be enough to raise suspicions• Glucagonoma should also be suspected in any patient with new onset of diabetes after age 60 +++ Rule Out + • Other causes of dermatitis• Other pancreatic islet cell tumors + • Elevated serum glucagon• Decreased serum amino acids• CT scan or MRI +++ When to Admit + • Severe symptoms + • Surgical resection of the primary tumor-Location of tumor determines procedure +++ Surgery + • Distal subtotal pancreatectomy and splenectomy (for head and body)• Pancreaticoduodenectomy (for head)• Debulking of metastases +++ Indications + • Whenever technically possible• Preoperative total parenteral nutrition should be administered for malnutrition +++ Medications + • Total parenteral nutrition• Somatostatin for symptomatic palliation• Streptozocin and dacarbazine for unresectable lesions +++ Prognosis + • Good with complete resection of the tumor• Palliation can be achieved with resection and debulking of metastatic disease +++ References ++Chastain MA. The glucagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci. 2001;321:306. [PubMed: 11370794] Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth