A rare tumor
A glucagonoma is a tumor of the pancreas that produces the hormone glucagon, which raises the level of sugar (glucose) in the blood and causes a distinctive rash. About 80% of glucagonoma's are cancerous. However, they grow slowly, and many people survive for 15 years or more after the diagnosis. The average age at which symptoms begin is 50. About 80% of people with glucagonomas are women. A few people have multiple endocrine neoplasias.
Symptoms and Diagnosis
High levels of glucagon in the blood cause the same symptoms like diabetes, such as weight loss and frequent, excessive urination. In addition, 90% of people have the very distinctive features of a chronic brownish-red skin rash (necrolytic migratory erythema) and a smooth, shiny, bright red-orange tongue. The mouth also may have cracks at the corners. The rash, which causes scaling, starts in the groin and moves to the buttocks, forearms, hands, feet, and legs.
The diagnosis is made by identifying high levels of glucagon in the blood and then locating the tumor by performing an abdominal CT scan followed by endoscopic ultrasonography. Magnetic resonance imaging (MRI) or a PET scan may be used if the CT scan does not show a tumor.
Glucagonoma is a type of neuroendocrine tumor (NET) that starts in the pancreas. The treatment you have depends on a number of things. This includes where the tumor is, its size, and whether it has spread (the stage).
But surgery isn’t always possible. Some glucagonomas may have already started to spread when you are diagnosed. Or you may not be well enough to have it. You continue to have treatment to help your symptoms if surgery isn’t an option.
Deciding which treatment, you need
A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT).
The treatment you have depends on:
- where the tumor is and its size
- whether it has spread
- your general health
- whether you have a rare inherited syndrome called multiple endocrine neoplasias 1 (MEN1)
Surgery is the only treatment that can cure a glucagonoma. The type of surgery you have depends on the size of the tumor, where it is, and whether it has spread to other parts of the body such as the liver.
Treatments to help with symptoms
You have treatments to help with symptoms before you have surgery. You might also have these treatments if you can’t have surgery to remove the tumour, or if it comes back after surgery.
Dr. Rakesh Rai. MS, FRCS, MD, CCT, ASTS Fellow.