ZocdocAnswersWhat is Glucagon Syndrome and should I be tested if I have a family history of it?

Question

What is Glucagon Syndrome and should I be tested if I have a family history of it?

My aunt died of Glucagon Syndrome when she was 33yrs old. The test came back with highly elevated levels after she had already died. Since I have many medical problems in common with her and am turning 29 soon I'd like to know more about it and if I should go ahead and get tested for it.

Answer

It sounds like your aunt may have had a tumor called a glucagonoma. There is not a specific diagnosis called Glucagon Syndrome, but this phrase may have been used to describe a glucagonoma. A glucagonoma is a very rare type of pancreatic tumor that releases abnormally high levels of the hormone glucagon. This hormone's main action is to increase sugar in the blood. People with this type of tumor experience a wide variety of symptoms including diarrhea, diabetes, and weight loss. In addition, a skin condition called necrolytic migratory erythema can develop which often is a sign that leads to the diagnosis. The way you test for this tumor is by measuring the levels of glucagon in your blood. Since the tumor is so rare, and not know to be genetically linked, testing for it would not be done routinely. Thus, you would have to ask for it from your doctor. I personally would not recommend getting tested unless you showed one of the cardinal symptoms (the rash, diabetes, weight loss) because the likelihood is of having it is low. The good news is that removal of the tumor can be curative. Thus, you should keep a regular schedule of seeing your primary care physician for annual physicals. This way your symptoms can be caught early. Good luck.

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