Learn About Glucagonoma
Glucagonoma is a very rare tumor of islet cells of the pancreas, which leads to an excess of the hormone glucagon in the blood.
Glucagonoma syndrome; 4D syndrome; Endocrine-secreting islet cell tumor - glucagonoma
Glucagonoma is usually cancerous (malignant). The cancer tends to spread and get worse if not treated.
This cancer affects some of the islet cells of the pancreas. As a result, the affected islet cells produce too much of the hormone glucagon.
The cause is unknown. Genetic factors play a role in some cases. A family history of the syndrome multiple endocrine neoplasia type I (MEN I) is a risk factor.
Symptoms of glucagonoma may include any of the following:
- Glucose intolerance (body has problem breaking down sugars)
- High blood sugar (hyperglycemia)
- Diarrhea
- Excessive thirst (due to high blood sugar)
- Frequent urination (due to high blood sugar)
- Increased appetite
- Inflamed mouth and tongue
- Nighttime (nocturnal) urination
- Skin rash on face, abdomen, buttocks, or feet that comes and goes, and moves around (necrolytic migratory erythema)
- Weight loss
In most cases, the cancer has already spread to the liver when it is diagnosed.
Surgery to remove the tumor before it spreads is usually recommended. Chemotherapy may be done when surgery is not possible.
MD Anderson
James Yao is an Oncologist in Houston, Texas. Dr. Yao is rated as a Distinguished provider by MediFind in the treatment of Glucagonoma. His top areas of expertise are Neuroendocrine Tumor, Pancreatic Islet Cell Tumor, Metastatic Insulinoma, Carcinoid Syndrome, and Pancreatectomy.
University Hospitals Medical Group Inc
Jeffrey Hardacre is a General Surgeon and a Surgical Oncologist in Cleveland, Ohio. Dr. Hardacre is rated as a Distinguished provider by MediFind in the treatment of Glucagonoma. His top areas of expertise are Pancreatic Cancer, Esophageal Cancer, Insulinoma, Pancreatectomy, and Pancreaticoduodenectomy. Dr. Hardacre is currently accepting new patients.
Regents Of The University Of California
Diane Simeone is a General Surgeon and a Surgical Oncologist in La Jolla, California. Dr. Simeone is rated as a Distinguished provider by MediFind in the treatment of Glucagonoma. Her top areas of expertise are Pancreatic Cancer, Familial Pancreatic Cancer, Pancreatic Ductal Adenocarcinoma, Pancreatectomy, and Small Bowel Resection. Dr. Simeone is currently accepting new patients.
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Approximately 60% of these tumors are cancerous. It is common for this cancer to spread to the liver. Only about 20% of people can be cured with surgery.
If the tumor is only in the pancreas and surgery to remove it is successful, people have a 5-year survival rate of 85%.
The cancer can spread to the liver. High blood sugar level can cause problems with metabolism and tissue damage.
Contact your provider if you notice symptoms of glucagonoma.
Summary: Multiple Endocrine Neoplasia type 1 (MEN1) is an autosomal dominant disease with a high degree of penetrance (\>80% of patients). It is caused by the presence of the MEN1 mutation located on chromosome 11q13. The prevalence of this mutation is estimated at approximately 1/30,000. This hereditary syndrome is characterized by the presence of tumours of the endocrine system (adenoma of the parathyroi...
Published Date: October 01, 2024
Published By: Sandeep K. Dhaliwal, MD, board-certified in Diabetes, Endocrinology, and Metabolism, Springfield, VA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Asban A, Patel AJ, Reddy S, Wang T, Balentine CJ, Chen H. Cancer of the endocrine system. In: Niederhuber JE, Armitage JO, Kastan MB, Doroshow JH, Tepper JE, eds. Abeloff's Clinical Oncology. 6th ed. Philadelphia, PA: Elsevier; 2020:chap 68.
National Cancer Institute website. Pancreatic neuroendocrine tumors (islet cell tumors) treatment (PDQ) - health professional version. www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq. Updated August 16, 2024. Accessed October 14, 2024.
Newey PJ, Thakker RV. Endocrine neoplasia syndromes. In: Melmed S, Auchus RJ, Goldfine AB, Rosen CJ, Kopp PA, eds. Williams Textbook of Endocrinology. 15th ed. Philadelphia, PA: Elsevier; 2025:chap 42.