The
glucagonoma syndrome is a rare disorder characterized by
weight loss,
necrolytic migratory erythema (NME), diabetes,
stomatitis, and
diarrhea. We identified 21 patients with the
glucagonoma syndrome evaluated at the Mayo Clinic from 1975 to 1991. Although NME and diabetes help identify patients with
glucagonomas, other manifestations of malignant disease often lead to the diagnosis. If the diagnosis is made after the
tumor is metastatic, the potential for cure is limited. The most common presenting symptoms of the
glucagonoma syndrome were
weight loss (71%), NME (67%),
diabetes mellitus (38%), cheilosis or
stomatitis (29%), and
diarrhea (29%). Although only 8 of the 21 patients had diabetes at presentation, diabetes eventually developed in 16 patients, 75% of whom required
insulin therapy. Symptoms other than NME or
diabetes mellitus led to the diagnosis of an
islet cell tumor in 7 patients. The combination of NME and
diabetes mellitus led to a more rapid diagnosis (7 months) than either symptom alone (4 years). Ten patients had
diabetes mellitus before the onset of NME. No patients had NME clearly preceding
diabetes mellitus. Increased levels of secondary
hormones, such as
gastrin (4 patients),
vasoactive intestinal peptide (1 patient),
serotonin (5 patients),
insulin (6 patients, clinically significant in 1 only), human
pancreatic polypeptide (2 patients),
calcitonin (2 patients) and
adrenocorticotropic hormone (2 patients), contributed to clinical symptoms leading to the diagnosis of an
islet cell tumor before the onset of the full
glucagonoma syndrome in 2 patients. All patients had metastatic disease at presentation. Surgical debulking,
chemotherapy,
somatostatin, and hepatic artery embolization offered palliation of NME, diabetes,
weight loss, and
diarrhea. Despite the malignant potential of the
glucagonomas, only 9 of 21 patients had
tumor-related deaths, occurring an average of 4.91 years after diagnosis. Twelve patients were still alive, with an average age follow-up of 3.67 years.