• Talia Burneikis
  • Vikram D. KrishnamurthyEmail author


Glucagonomas are rare functional neuroendocrine tumors of the pancreas that secrete excessive glucagon, which presents with weight loss, glucose intolerance/diabetes, and necrolytic migratory erythema. Less common symptoms include diarrhea, venous thromboembolic disease, and neuropsychiatric manifestations. Most cases are sporadic, but up to 20% may be associated with multiple endocrine neoplasia syndrome type 1 (MEN1). Initial treatment is supportive and involves glycemic control and improving nutritional status. When disease burden is amenable, resection of the primary tumor and debulking of metastases are indicated.


Glucagonoma Hyperglycemia Neuroendocrine tumor Multiple endocrine neoplasia MEN1 


  1. 1.
    Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV. The glucagonoma syndrome. Clinical and pathologic features in 21 patients. Medicine (Baltimore). 1996;75(2):53.CrossRefGoogle Scholar
  2. 2.
    Dromain C, de Baere T, Baudin E, Galline J, Ducreux M, Boige V, Duvillard P, Laplanche A, Caillet H, Lasser P, Schlumberger M, Sigal R. MR imaging of hepatic metastases caused by neuroendocrine tumors: comparing four techniques. AJR Am J Roentgenol. 2003;180(1):121.CrossRefGoogle Scholar
  3. 3.
    Khashab MA, Yong E, Lennon AM, Shin EJ, Amateau S, Hruban RH, Olino K, Giday S, Fishman EK, Wolfgang CL, Edil BH, Makary M, Canto MI. EUS is still superior to multidetector computerized tomography for detection of pancreatic neuroendocrine tumors. Gastrointest Endosc. 2011;73(4):691.CrossRefGoogle Scholar
  4. 4.
    Nauck C, Ivancevic V, Emrich D, Creutzfeldt W. 111In-pentetreotide (somatostatin analogue) scintigraphy as an imaging procedure for endocrine gastro-entero-pancreatic tumors. Z Gastroenterol. 1994;32(6):323–7.PubMedGoogle Scholar
  5. 5.
    Fraker DL, Norton JA. The role of surgery in the management of islet cell tumors. Gastroenterol Clin N Am. 1989;18(4):805.Google Scholar
  6. 6.
    Karabulut K, Akyildiz HY, Lance C, Aucejo F, McLennan G, Agcaoglu O, Siperstein A, Berber E. Multimodality treatment of neuroendocrine liver metastasis. Surgery. 2011;150(2):316–25.CrossRefGoogle Scholar
  7. 7.
    National Comprehensive Cancer Network (NCCN). NCCN Clinical practice guidelines in oncology. Accessed on 27 Feb 2016.

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Endocrine SurgeryThe Cleveland ClinicClevelandUSA

Personalised recommendations