A retrospective study of 14 cases of glucagonoma with literature review

Abstract

Objective To improve the clinical understanding of glucagonoma. Methods A total of 14 cases of glucagonoma were admitted to our hospital, and the clinical features of these cases were retrospectively analyzed. Results (1) The female/male case ratio was 1∶1. (2) The median age of onset was 47 years (range 33-61), while the median age at diagnosis was 50 years (range 33-64). (3) The most common initial presentation was necrolytic migratory erythema (7/14, 50%). (4) Hyperglycemia was the most common presentation at the time of diagnosis (14/14, 100%), followed by necrolytic migratory erythema (13/14, 92.9%). (5) The misdiagnosis rate before admission to our hospital was as high as 85.7% (12/14), and the most common misdiagnosis was eczema (7/12, 58.3%). (6) The median time from onset of probable symptoms to diagnosis was 4 years. (7) The median plasma glucagon level at diagnosis was 798 pg/ml (range 200-2 853). (8) The median length of the longest tumor diameter of the pancreatic tumors at diagnosis was 4.0cm (range 2.2-8.0). (9) 57.1% of primary pancreatic glucagonomas localized in the tail of the pancreas. (10) 57.1% of patients (8/14) had metastases at the time of diagnosis. Conclusions (1)Necrolytic migratory erythema is a relatively sensitive and specific symptom for diagnosis of glucagonoma. (2)The diagnosis for glucagonoma was often delayed, and misdiagnosis was common. (3)Most glucagonoma patients had metastases at the time of diagnosis. (Chin J Endocrinol Metab, 2018, 34: 909-914) Key words: Glucagonoma; Necrolytic migratory erythema; Diabetes mellitus; Neuroendocrine tumor.