Glucagonoma and Glucagonoma Syndrome: One Center's Experience of Six Cases.

Purpose: Glucagonoma is an extremely rare neuroendocrine tumor arising from pancreatic islet cells. Although patients with glucagonoma manifest multiple typical symptoms, early diagnosis remains difficult due to the scarcity of this disease. Methods: In this study, we retrospectively screened the database of the pancreas center of Nanjing Medical University. A total of six cases diagnosed as glucagonoma during the past 17 years were included. Their clinical characteristics and treatments were reviewed. Results: The six patients consisted of four females and two males. Their median age at diagnosis was 48.7 years (range 35-77). The time from onset of symptoms to diagnosis of glucagonoma ranged from 1.3 months to >10 years. Common symptoms included necrotizing migratory erythema shown in six of six patients (100%), diabetes mellitus in five of six patients (83%), stomatitis in four of six patients (67%), and weight loss in four of six patients (67%). Plasma glucagon levels were elevated in all patients (range 245.6-1132.2 pg/mL; n  < 200), and significantly declined after surgery (range 29-225.1 pg/mL; n  < 200). Imaging studies revealed that three of six patients had metastasis at the time of diagnosis. All patients received surgical resection. The primary lesion, liver metastases, and involved organs were resected in all patients if present. The mean survival time was 5.7 years (range 3-10.4) from diagnosis and four of six patients died of this disease by the time of follow-up. Conclusion: Our data suggest surgery is effective for symptom relief and can control the progress of glucagonoma. Early diagnosis and surgery are crucial for glucagonoma.