Glucagonoma is an extremely rare
neuroendocrine tumor that arises from pancreatic islet alpha cells. Although
glucagonoma is usually accompanied by a variety of characteristic clinical symptoms, early diagnosis is still difficult due to the scarcity of the disease. In this study, we present the cumulative experiences, clinical characteristics and treatments of seven patients diagnosed with
glucagonoma during the past 10 years at the First Affiliated Hospital of Xi'an Jiaotong University. The seven patients in our cohort consisted of six females and one male with an average diagnosis age of 40.1 years (range 23-51). The average time from onset of symptoms to diagnosis of
glucagonoma was 14 months (range 2-36 months). All the patients visited dermatology first for
necrolytic migratory erythema (NME) 7/7 (100%), and other presenting symptoms included
diabetes mellitus (DM) 4/7 (57%),
stomatitis 2/7 (28%),
weight loss 4/7 (57%),
anemia 4/7 (57%),
diarrhea 1/7 (14%), and DVT1/7 (14%). Plasma
glucagon levels were increased in all patients (range 216.92-3155 pg/mL) and declined after surgery. Imaging studies revealed that four of seven patients had liver
metastasis. Six of seven patients received surgical resection, and all of them received
somatostatin analog
therapy. Symptoms improved significantly in 6 out of 7 patients. Three of seven patients died of this disease by the time of follow-up. Our data suggest that if persistent NME is associated with DM and high
glucagon levels, timely abdominal imaging should be performed to confirm
glucagonoma. Once diagnosed, surgery and
somatostatin analogs are effective for symptom relief and
tumor control.