Stephen J. Schueler, M.D.

Overview Symptoms

Glucagonoma Overview

What is a glucagonoma?
A person with a glucagonoma has a very rare malignant tumor that can develop in the islet cells of the pancreas. About 50% of malignant glucagonomas have spread outside of the pancreas at the time of diagnosis. These tumors are discovered because they release the hormones insulin and glucagon. These hormones are responsible for many of the symptoms of glucagonoma syndrome. Another important feature of glucagonoma syndrome is a very high risk for pulmonary embolism.

What are the symptoms of a glucagonoma?
The symptoms of a glucagonoma include diarrhea, excessive thirst, inflammation of the mouth and tongue, nighttime enuresis, frequent urination, and a skin rash on the face, abdomen, buttocks and legs that comes and goes.

How does the doctor treat a glucagonoma?
The treatment for a glucagonoma includes surgery to remove the tumor. Medications that have been used to treat glucagonoma include octreotide (Sandostatin), doxorubicin (Adriamycin, Rubex), fluorouracil (Adrucil), streptozocin (Zanosar), and dacarbazine (DTIC-Dome).

Continue to Glucagonoma Symptoms

Last Updated: Jul 22, 2010 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Glucagonoma References
  1. Falchetti A, Marini F, Brandi ML Multiple Endocrine Neoplasia Type 1. 2005 Aug 31 [updated 2010 Mar 2]. In: Pagon RA, Bird TC, Dolan CR, Stephens K, editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993 [20301710]
  2. Henopp T, Anlauf M, Schmitt A, Schlenger R, Zalatnai A, Couvelard A, Ruszniewski P, Schaps KP, Jonkers YM, Speel EJ, Pellegata NS, Heitz PU, Komminoth P, Perren A, Kloppel G. Glucagon cell adenomatosis: a newly recognized disease of the endocrine pancreas. J Clin Endocrinol Metab. 2009 Jan;94(1):213-7. [18957496]
  3. Okauchi Y, Nammo T, Iwahashi H, Kizu T, Hayashi I, Okita K, Yamagata K, Uno S, Katsube F, Matsuhisa M, Kato K, Aozasa K, Kim T, Osuga K, Nakamori S, Tamaki Y, Funahashi T, Miyagawa J, Shimomura I. Glucagonoma diagnosed by arterial stimulation and venous sampling (ASVS). Intern Med. 2009;48(12):1025-30. [19525592]
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